An elderly person, aged 60 years, presented with pain abdomen localized to the epigastric region, of one year duration. There was a past history of a single episode of passing black-colored tarry stools and hematemesis. Ultrasonography (USG) of abdomen re-ported a suspected duodenal / periampullary growth. Biopsy per-formed was inconclusive. Contrast enhanced computerized tomo-graphy (CECT) scan abdomen suggested suspected submucosal leiomyoma of duodenum with intrahepatic biliary distension. Whipple’s procedure was performed which recovered a growth measuring 6x5x5cms arising in periampullary region of second part of duodenum. Pancreatic duct and common bile duct (CBD) were dilated. The histopathological findings were suggestive of neuroendocrine tumor of duodenum. The diagnosis of gangliocyt-ic paraganglioma was confirmed by immunohistochemical stains.