We report a patient in whom heterotopic pancreatic tissue was found within the ampulla and treated by ampullectomy. Only 16 cases of pancreatic tissue located at the ampulla of Vater have been reported in the literature and pancreaticoduodenectomy has been performed in more than half the cases because malignancy was suspected preoperatively, reflecting a rather aggressive surgical attitude. An accurate preoperative evaluation thus appears decisive to avoid unnecessary radical surgery. Endoscopic treatment of ampullary tumors is emerging as a viable alternative to surgical treatment. Local surgical excision is a reasonable option to endoscopic treatment. The possibility of heterotopic pancreatic tissue, though rare, should be included in the differential diagnosis of papillary tumors. Histologic confirmation (frozen or delayed) is mandatory before attempting such major surgery.