The appendix is the most common location for carcinoid tumors within the ga
strointestinal tract. Carcinoid tumors of the ampulla of Vater is an extrem
ely rare entity; only 71 cases of carcinoid of the ampulla of Vater have be
en reported in the literature to date. The clinical picture is non-specific
, and endoscopy is the main diagnostic procedure; but large and deep biopsi
es 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, ther
apeutic challenge and results.