From 1964 to 1977 a total of 50 patients were operated on for carcinoma of the ampulla of Vater, the second most frequent tumor of the periampullar region. The cardinal symptom was jaundice. The diagnostic procedures of high value were roentgenographic examination of the upper gastrointestinal tract with the use of barium, also hypotonic duodenography, sonography, and percutaneous transhepatic cholangiography (ERCP, angiography). Curative treatment was possible by duodenopancreatectomy, with a resection rate of 74%. The 5-year survival rate was 27% with a postoperative death rate of 13.5%.