A 23-year-old female, previously diagnosed with Polycystic Ovarian Syndrome( PCOS) and pituitary macroprolactinoma presented with drowsiness, confusion and profuse sweating developing over a period of one day. Parents revealed that she was having a hallucinatory behavior that is both auditory and visual, in the preceding two weeks. She did not have fever, seizures or symptoms suggestive of meningism. There was recent increase in appetite with significant weight gain. Parents could not recall any improvement of symptoms following the meals. She neither smoked or consumed alcohol and did not consume any psychoactive substances. She did not travel recently. At the age of 16 years, she was evaluated for hirsutism and oligomenorrhoea by a gynaecologist and was found to have bilateral, multiple ovarian cysts on ultrasound scan. She was diagnosed with PCOS and was advised on lifestyle modification including weight reduction. Serum Prolactin, Thyroid Stimulating Hormone (TSH) and total Testosterone levels were within normal range at that time. Four years later, she was re-evaluated by an endocrinologist for persistent oligomenorrhoea and hirsutism. She was diagnosed to have a pituitary microprolactinoma (7 mm × 5 mm) with a serum Prolactin level of 347 ng/mL( 4.04–15.2 ng/mL) while rest of her pituitary hormones, total Testosterone and Dehydroepiandrosterone (DHEA) levels were within normal range. Bilateral polycystic ovaries were noted during the repeat ultrasound scan of abdomen and pelvis. An asymptomatic, non-obstructing left side renal calculus was noted during this scan by the radiologist, but it was not further evaluated. She was started on Cabergoline 0.5 mg weekly but was lost to follow up thereafter. There was no family history of established familial multiple endocrine disorders but her paternal grandmother and aunt had a history of recurrent renal calculi. She was the first offspring of a non-consanguineous marriage. Clinical examination revealed an obese lady with a Body mass index(BMI) of 32 kg/m2. She had signs of hyperandrogenism i.e.; hirsutism and acne vulgaris. There were multiple, skin coloured, dome shaped, smooth surfaced papules of 3-5 mm involving the anterior chest and abdominal wall suggestive of collagenomas which was later confirmed histologically as well. Her Glasgow Coma Scale (GCS) score was 9/15. Patient was sweating profusely. Pulse rate was 110 beats per minute. Blood pressure was 120/80 mmHg. Neurological examination revealed sluggishly reactive pupils. Fundal examination was normal. The tendon reflexes were exaggerated with up going planter reflexes. Abdominal examination revealed a nontender, smooth surfaced, firm, moderate hepatomegaly. Rest of the clinical examination was normal. Her Random plasma glucose level was 23.3 mg/dL on admission. She was treated with intravenous 20% dextrose boluses followed continuous infusion. But the patient continued to develop recurrent, severe hypoglycaemic episodes. Due to the prolonged reduced conscious level during the episodes of hypoglycaemia, elective intubation was carried out to protect the airway and to prevent aspiration. Initial laboratory evaluation including full blood count and inflammatory markers were within normal parameters. Non-contrast Computed Tomography (CT) of brain was normal. Cerebrospinal fluid (CSF) analysis was normal except for very low sugar levels. Serum insulin level was 300 mU/L (fasting level < 25 mU/L) with a c-peptide level of 15.63 ng/mL (fasting level 0.78–1.89 ng/mL) which was drawn during an episode of confirmed severe hypoglycaemia. Toxicology screening did not reveal presence of oral hypoglycaemic agents. Her serum fasting gastrin level 70 pg/mL. Prolactin was 11,716 mU/L (300 – 500 mU/L). Serum Calcium (corrected to an albumin level of 3.2 g/dL) was 16.1 mg/dL(8.6–10.3 mg/dL) and serum phosphate level was 1.3 mg/dL(3–4.5 mg/dL). Intact parathyroid hormone (PTH) level was 329.7 pg/mL (18.4–80 pg/mL). 24-h urinary calcium excretion was 828.82 mg/dL (100 -300 mg/24 h). The findings were suggestive of endogenous hyperinsulinaemic hypoglycaemia and primary hyperparathyroidism. Thyroid stimulating hormone(TSH), Thyroxine (fT4), Cortisol, Growth hormone and Follicular stimulating hormone (FSH), Luteinizing hormone (LH), total Testosterone and DHEA levels were within the respective normal range. Our patient was having recurrent, endogenous hyperinsulinaemic hypoglycaemia and primary hyperparathyroidism with multiple cutaneous collagenomas in the background of pituitary microadenoma and family history of recurrent nephrolithiasis. A unifying clinical diagnosis of MEN 1 syndrome appeared to be the likely explanation for current presentation. The patient was extubated following a protracted recovery of conscious level with few residual neurological sequalae. But she continued to have frequent severe hypoglycaemic episodes even while on continuous intravenous 20% dextrose 100 mL/h infusions, Diazoxide 100 mg 8 hourly, Octreotide 100 µg 6 hourly, Growth hormone 2 mg daily and frequent high glycaemic index carbohydrate meals. Continuous 20% dextrose infusion with intermittent 50% dextrose boluses and hourly capillary blood glucose monitoring was required to maintain normoglycaemia. Diazoxide and Growth hormone were discontinued after one month due to poor response. Octreotide was continued considering its inhibitory action on pancreatic insulin secretion. None of these pharmacological treatments were able to completely ameliorate the hypoglycaemic episodes. Hypercalcemia was managed with volume expansion, a single dose of Zoledronic acid 4 mg and Cinacalcet 30 mg daily. Her hospital stay was further complicated with numerous episodes of ventilator associated pneumonia, infected vascular accesses and recurrent urinary tract infections. Repeated cultures isolated both bacterial and fungal organisms which required prolonged courses of systemic antibiotics and antifungal therapy. Extensive radiological imaging was done to localize the tumours. Contrast enhanced Computed Tomography (CECT) scan of abdomen and pelvis revealed three focal lesions in liver segments II, IV and VII with enhancement in arterial phase and washout in the venous phase, suggestive of hypervascular lesions. Pancreatic imaging was normal. There was no evidence of renal calculi or suprarenal masses. A follow up Magnetic resonance imaging (MRI) of Abdomen reported that the focal liver lesions are more in favour of benign nature i.e. two focal lesions in segments II and IV are likely to be haemangiomas while the lesion is segment VII is suggestive of Focal nodular hyperplasia. It was noted that the pancreas is atrophic without presence of mass lesions or duct dilatation. Due to diagnostic uncertainty, a whole body fluorodeoxyglucose Positron Emission Tomography—Computed Tomography (FDG PET/CT) scan was performed. It did not show any metabolically active lesions in liver or pancreas. MRI brain and neck revealed T2/FLAIR (Fluid-attenuated inversion recovery) hyperintensity with diffusion restriction in bilateral globus pallidi, left parieto-occipital cortex and bilateral temporal lobe cortices medially, which was in favour of hypoglycaemic encephalopathy. The pituitary gland was normal in size with a hypointense lesion in left lobe that was consistent with her history of pituitary microadenoma. During the localization of suspected the parathyroid adenoma, soft tissues in neck region was not visualized properly either with ultrasound imaging, CECT or MRI imaging which was attributed to the obese body habitus of the patient. Hence further evaluation with nuclear imaging was suggested but the necessary nuclear isotope imaging facility to localize parathyroid adenoma was not available temporarily in our institute during that time. Since none of the imaging modalities were able to localize the insulinoma, a selective arterial calcium stimulation test (SACST) was performed. It was able to localize the insulinoma to the distal body of the pancreas. Following successful localization of insulinoma, feasibility of surgical resection was discussed in the multidisciplinary team meeting (MDT). MDT concluded that the patient was having high risk of surgery related mortality due to ongoing sepsis and resistant hypoglycaemia. Percutaneous chemoembolization appeared to be a favorable treatment option at that time. Chemoembolization was performed following the successful identification of vascular supply to the insulinoma with intraprocedural angiography. The following day, patient developed severe acute pancreatitis complicated with acute respiratory distress syndrome and required intensive care. Chemoembolization appeared to be unsuccessful since the patient continued to have hypoglycaemic episodes. MDT decided that the patient requires an open surgery to resect distal pancreatic insulinoma. Following extensive preoperative optimization and planning, an open distal pancreatectomy with splenectomy was performed. Histological assessment revealed a well differentiated pancreatic islet cell tumour. Following surgery, hypoglycaemia improved dramatically with normalization of blood glucose, serum insulin and c-peptide levels. Three weeks after the first surgery, patient underwent standard four gland exploration of parathyroid glands which revealed two large parathyroid glands on left side. Four gland parathyroidectomy was performed followed by implantation. Later they were histologically confirmed as parathyroid adenomas. Following satisfactory clinical and biochemical recovery, patient was discharged home after 6 months from her admission. Prior to discharge, she underwent a formal cognitive assessment which showed mild deficits in executive functioning, language, memory and processing speed domains. She was arranged follow up with psychiatric services and occupational therapy. Follow-up serum insulin, c-peptide, intact PTH, and calcium levels were found to be within the normal range. Two months from discharge, she presented with brief episode of right side, tonic clonic, focal seizure with preserved awareness. Blood glucose, serum insulin, c -peptide, intact PTH, and calcium levels were found to be normal. MRI brain was repeated which revealed an area of gliosis in left side temporal lobe. The area of gliosis corresponded with the MRI brain that was performed during the previous admission, which demonstrated the typical features of hypoglycaemic encephalopathy. She was evaluated by a neurologist and was diagnosed with a remote symptomatic seizure. She was started on antiepileptic medication to prevent recurrent seizures and progressive brain injury. Currently she is being follow-up regularly and continues to be on Cabergoline for prolactin secreting pituitary microadenoma. Biochemical screening of first-degree family members was also done which was found to be negative, but we were not able to screen the two relatives who had history of recurrent nephrolithiasis. Unfortunately, facilities to carry out the genetic analysis is not available locally. The endocrine team is currently in discussion with a research centre in United Kingdom to arrange the genetic studies for the patient and the family members.