Schwannomas are neurogenic tumors that arise from Schwann cells in the neural sheath. Gastrointestinal schwannomas occur most often in the stomach, followed by the colon and the rectum. Duodenal schwannomas are rare amongst mesenchymal tumors of the gastrointestinal tract and only a few cases have been reported up to the current date with an incidence of approximately 2%-6%. Duodenal Schwannomas do not have characteristic imaging features thereby cannot be easily differentiated from other submucosal and adjacent extraluminal neoplasms. We present a case of a 76-year old male patient that presented to our hospital with abdominal pain and was diagnosed after an upper gastrointestinal endoscopy with an ampullary duodenal neoplasm that proved to be a periampullary duodenal Schwannoma on histopathology. Duodenal Schwannomas although rare should be considered in the differential diagnosis of ampullary neoplasms.