Abstract
Somatostatinoma is a neuroendocrine tumor; its incidence in the duodenum around the ampulla of Vater is rare, and it is often not associated with secretory manifestation. The authors report a case of a female with an ampullary (Vater) tumor displaying neuroendocrine nuclear features, psammoma bodies, and the positivity for immunohistochemical panel of neuroendocrine tumors. The patient was presented with some features compatible with somatostatin secretion-associated syndrome; albeit with normal serum levels of the hormone. Initial attempt of complete resection failed, and the involved margins were revised with a subsequent surgery, and the patient showed an uneventful course on follow-up for two years.