Malignancies of the biliary tree, particularly the extrahepatic bile ducts, remain difficult clinical problems. Because experience with these uncommon tumors has been limited to a small number of centers, meaningful clinical trials have been difficult to perform. Complete resection remains the most effective therapy, but is usually possible in the minority of patients. Palliating the effects of biliary obstruction is thus often the primary therapeutic goal. Chemotherapy and radiation therapy have not been proven to reduce the incidence of recurrence after resection nor to improve survival in patients with unresectable disease. This review focuses on cholangiocarcinoma of the extrahepatic bile ducts.