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