We report the case of a 32 years old male, father of a 4 years old son, working as a carpenter, who presented to the surgery outpatient department of a tertiary level hospital in Nepal, with a 2 year history of urgency, increased frequency of micturition, constipation. However, the symptoms increased over time and there was perianal pain for 2 months, pain after ejaculation for 1 month. The International Prostatic Symptom Score was 14. The patient's history was negative for fever, hematuria, hematospermia, per rectal bleeding. There were no any comorbidities and significant family history. The patient was not under any medications. Findings on his arrival were as follows: body height, 172 cm; body weight, 66 kg - BMI was 22.3 (normal weight).; blood pressure, 120/70 mmHg; pulse, 80 beats/min; body temperature, 37.3 °C; no anemia and icterus in the palpebral conjunctiva; no edema in the legs, no cyanosis; no any palpable lymph nodes breathing sounds were clear/no secondary noises; no heart murmurs. No any other significant examination findings were present per abdomen. Per rectal examination revealed an anal fissure measuring 0.5 cm × 0.2 cm at 5 o'clock position. Hematological tests were sent and urinalysis was performed, the report of which were within normal limits. Urine culture also didn't show growth of any organism. Ultrasonography (USG) of Abdomen and Pelvis () couldn't find the right kidney and revealed few cystic lesion in the right pelvis close to prostate suggestive of dilated seminal vesicle, diffuse echogenic debris in urinary bladder suggestive of cystitis with normal sized prostate weighing approximately 11 g with normal outline and echo pattern. The patient was then managed conservatively with oral antibiotics, disodium hydrogen citrate, aceclofenac and selective antagonist of post-synaptic alpha-adrenoreceptors and with ointment nitroglycerine and isphagulla. The patient's symptoms were still not relieved for which a sigmoidoscopy () was done as further diagnostic procedure which showed a globular submucosal lesion in the rectum. However, biopsy was not taken. Computed Tomography (CT) of Abdomen and Pelvis (A-D) were done which revealed solitary, left, functioning kidney, with no evidence of the right renal unit with the presence of a non-enhancing cystic lesion measuring 69 × 71 mm in recto sigmoid region more on the right side of the pelvis posterior to the urinary bladder. The seminal vesicles bilaterally were found to be enlarged. Post contrast study showed no enhancement, no enhancing septa with the lesion showing narrow tubular structure with blind area in proximal part. Post CT USG showed bean shaped cystic lesion in the posterior part of urinary bladder with significant post void urine (93 ml). An MRI abdomen and pelvis (A-C) was also done which showed:Dilated lobular and tortuous cystic lesion in the Right side of pelvic cavity, the maximum diameter of which was 4 cm, extending along the right side pelvic wall up to the level of aortic bifurcation and opening into the prostatic urethra and showing wall enhancement in post contrast images. The lesion was seen to be abutting prostate inferiorly, urinary bladder and small bowel loops anteriorly Another 4.6 × 3.1 × 2.5 cm sized similar dilated, tubular and tortuous cystic lesion in the left side of pelvic cavity, with diameter of 1.4 cm, opening in prostatic urethra. Prostate normal in size, outline and parenchymal signal intensity. Non-visualization of Right kidney. Left kidney measuring 12.5 × 6.7 cm in size However, MRI did not reveal any rectal mass or wall thickening of the rectum. Urethrocystoscopy was done which showed no any lesions in urethra or bladder with a protruding mass at right lateral trigonal region which was partially obstructing the bladder neck. Imaging findings along with cystoscopic examination lead to the diagnosis of Zinner's Syndrome. Pelvic exploration with aspiration of cystic fluid accompanied by open surgical excision of cystic structure was then done by the consultant urologist. Operative finding include enlarged bilateral seminal vesicles, right sided about 5 cm in diameter left sided around 2 m in diameter; with 70 ml of cystic fluid in the right seminal vesicle. The aspirated cystic fluid was sent for microbiological examination which eventually came negative while the excised tissue was sent for histopathological assessment which revealed cyst wall lined by pseudostratified columnar epithelium. Few of the epithelial cells show reddish brown lipofuscin granules in the cytoplasm. Wall of the cyst comprises of fibromuscular tissue with chronic inflammatory cell infiltrates. These findings are consistent with Seminal Vesicle Cyst. The post-operative period was uneventful and the patient did not experience any further genitourinary discomfort. The patient was then discharged on 3rd post-operative day and was symptomatically better and hemodynamically stable at time of discharge.