Adenocarcinoma of the ampulla of Vater represents about 10% of periampullary neoplasms. This tumor is slow-growing, often amenable to radical surgical resection, and associated with a better prognosis than the other neoplasms which arise in the periampullary area. Correct identification of ampullary carcinoma may be difficult but is essential because of its better prognosis. A review of 31 patients with adenocarcinoma of the ampulla of Vater treated at our institution reveals that the classical history of fluctuating jaundice and the well known findings of a palpable gallbladder and occult blood in the stool may not be present. The treatment of choice for ampullary carcinoma in the absence of hepatic or distant metastases is pancreaticoduodenectomy. Our operative mortality for pancreaticoduodenectomy is 16%, and the five-year survival rate is 32%. The role of palliative procedures, the operative complications, and the survival rates are discussed.