Periampullary malignancy is the term utilized for neoplasms emerging from the head of pancreas, ampulla of Vater,
distal bile pipe and periampullary distinct of duodenum. Of these, the pancreatic adenocarcinoma conveys the most
noticeably awful anticipation with greater part of mortalities.Studies have shown that pancreatic carcinoma has the most
limited middle endurance of 17.1 months of all periampullary carcinomas. Herein, we present a case of a 63-year-old
male patient without any history of trauma or important previous symptoms presented to the tertiary care hospital with
compliance of sudden weight loss and abdominal pain over the preceding 3 months. On examination he was deeply
jaundice. His laboratory and radiological examination revealed periampullary adenocarcinoma. This individual had
successfully underwent Whipple procedure and followed by Chemotherapy. This case is being presented not only the
cancer is rare but also to understand and improve better clinical insight on this rare disease