The present paper reported a case of a carcinoma that probably developed from the peribiliary gland within the ampulla of Vater based on the histopathological findings of the resected specimens. A 49-year-old female became aware of epigastralgia and was diagnosis of stenosis of duodenal 2nd portion. There were no malignant findings on gastrointestinal endoscopy and computed thomography. Endoscopic retrograde cholangiopancreatography revealed no tumor in the main pancreatic duct or the common bile duct or ampulla of Vater. Pylorus preserving panctreaticoduodenectomy was performed with a diagnosis of duodenal stenosis of unknown cause. The histopathological findings revealed that a moderately to poorly differentiated adenocarcinoma originating near the peribiliary gland in the ampulla of Vater was extensively distributed in the submucosal layer of the duodenum. Based on these findings, a diagnosis of a carcinoma of the ampulla of Vater arising from the peribiliary gland was most likely suspected. Judging from the generally known development and extension of carcinoma of the ampulla of Vater, the current case appeared to be a very rare one.