The appendix is the most common location for carcinoid tumors within the gastrointestinal tract. Carcinoid tumors of the ampulla of Vater is an extremely rare entity; only 71 cases of carcinoid of the ampulla of Vater have been reported in the literature to date. The clinical picture is non-specific, and endoscopy is the main diagnostic procedure; but large and deep biopsies are often needed to verify the histological nature of the tumor. Surgery is the treatment of choice. We report a new case of this rare tumor, and a review of the literature concerning the clinical findings, diagnosis, therapeutic challenge and results.