The clinical-pathological spectrum of ampullary cancer in 18 patients studied at the Department of Gastroenterology (Hospital Oncológico Padre Machado) during the last decade (1980-88) is presented. Involvement of the extrahepatic bile ducts was due to cancer of the ampullary region. In all cases diagnosis was confirmed by pathology. The value of ERCP is presented and the need to set apart this tumors from carcinoma of the head of the pancreas is emphasized. None of the nine cases treated with radical resection (Whipple) had operative mortality, and although follow-up for all cases is not long enough, two patients are alive and free of disease, five years after surgery. The value of prosthesis and biliary drainage for palliation is commented upon. Emphasis is placed on the need of using the term ampullary cancer in its proper connotation. Criteria for differentiating periampullary tumors (specially cancer of the head of the pancreas) are presented.