Hypothesis: To our knowledge, few individual surgeons and only a handful of institutions have gained a meaningful experience with the treatment of adenocarcinoma of the extrahepatic bile ducts or cholangiocarcinoma. The purpose of this study was to critically evaluate the experience of a single center in the treatment of these tumors. Design: Retrospective cohort study with a median follow-up of 48 months. Setting: Department of surgery at a university referral center. Patients: Seventy-seven patients with biopsyconfirmed adenocarcinoma of the extrahepatic bile ducts evaluated and treated between January 1980 and February 1998. Main Outcome Measures: Prognostic variables, resectability rates, morbidity, and survival. Results: Thirty-eight male and 39 female patients were studied (median age, 71 years). Twenty-three patients (30%) underwent curative resections, 32 patients (41%) underwent palliative surgery, and 22 patients (29%) received nonoperative therapies. The 30-day perioperative morbidity rate was 18%, and mortality was 6%. Overall median survival was II months; 4 months for patients receiving nonoperative therapy; 8 months for patients recieving palliative surgery; and 72 months for curative resection. Five-year survival rates were 23%, 0% 10%, and 55%, respectively. Curative resection was the only prognostic variable to have a statistically significant effect on survival. Conclusions: Curative resection could be achieved in approximately one third of patients who had cholangiocarcinoma, and should be the goal of treatment. Survival is significantly improved in those patients who are considered to have resectable tumors and who undergo removal of all gross disease. Palliative surgical treatments also revealed a survival advantage over nonoperative therapies.