We report a case of a 22 years old pregnant female medically free, referred to us from her Obstetrician when she was complaining of abdominal pain and jaundice. Abdominal US confirmed a single intrauterine pregnancy at 28th week of gestation with appropriate growth for date and showed dilated intrahepatic ducts otherwise it was inconclusive due to the gravid uterus (). Her blood investigations showed a picture of cholestatic jaundice, and all other labs were within normal. So, we decided to proceed with a Magnetic resonance cholangiopancreatography (MRCP) which showed dilatation of both of the CBD (measuring 0.9 cm) & pancreatic duct, as well as an ampullary mass measuring 2 cm (). Later on, Endoscopic retrograde cholangiopancreatography (ERCP) with shielding of the abdomen to protect the fetus from radiation revealed an ampullary and distal CBD strictures. A punch biopsy was taken & the CBD was stented. The histopathology came as invasive adenocarcinoma & full metastatic work up was done and did not reveal any metastatic lesions. So, surgery was the best available option with the best possible outcome but we were reluctant to delay the surgery to ascertain the viability of the fetus. At 34th week of gestation induction of labor was done, both mother and the baby did well and were discharged home on 2nd day postpartum. The mother was readmitted one week later & full body CT scan repeated & there was no vascular invasion or distant metastasis. Therefore, we proceeded with pancreaticoduodenectomy. A laparotomy incision was done, intraoperative examination of the abdomen revealed; a palpable mass at the ampulla of Vater and the stent was felt in the CBD and duodenum, a bulky uterus as the patient was still in the postpartum period. There was no vascular invasion, peritoneal deposits or any other distant metastasis. For the pancreatojejunostomy anastomosis, a two-layer end-to-side duct-to-mucosa approach was adopted. The pancreatic duct was stented to divert the pancreatic secretions away from the anastomosis. Then the hepaticojejunostomy was done in an end-to-side fashion followed by the gastrojejunostomy. The Patient had uneventful postoperative course and was discharged 1 week after her surgery. Histopathology came as poorly differentiated invasive adenocarcinoma of ampulla of Vater with negative resection margins. Three out of thirteen lymph nodes revealed metastatic involvement so she received six cycles of adjuvant chemotherapy which she tolerated well. Upon 6 years follow up, computed tomography (CT) and positron emission tomography (PET) scans were normal with no evidence of recurrence.