Carcinoid tumors of the ampulla of Vater are extremely rare, and due to their location jaundice in approximately 2/3 of patients. A previously healthy 58-year-old man suffered from indigestion for one year. An abdominal CT and endoscopic ultrasonography showed a relatively well-enhancing mass (2.5 cm in size) at the ampulla of Vater and dilatation of the bile ducts. A duodenoscopy showed a luminally protruding mass, with preserved mucosa, at the ampulla of Vater. So a radical pancreatoduodenectomy was performed. From the operation a 2.3x2.3x2.0 cm-sized submucosal tumor at the ampulla of Vater was found. The cut surface of the tumor had a well circumscribed yellowish fine granular appearance, with a pushing tumor border. Histopathologically the tumor revealed tubular, trabecular and ribbon-like arrangements of tumor cells. The tumor cells had hyperchromatic round nuclei, with coarsely clumped chromatin and indistinct nucleoli. The neoplastic cells were diffusely and strongly immunoreactive for synaptophysin, chromogranin and CD56. We report the experience of a carcinoid tumor at the ampulla of Vater.