Case histories of 47 patients with histologically confirmed carcinoma of extrahepatic bile ducts presenting to M. D. Anderson Hospital and Tumor Institute over a 30-year period were studied. The disease was more common in males during the sixth and seventh decades of life. A third of the patients had history of cholelithiasis. Four patients had prior history of chronic ulcerative colitis and one patient had congenital choledochal cyst prior to development of cancer of the bile duct. Jaundice was the earliest and the most common presenting sign. The tumor was located in the common bile duct more often than in the hepatic ducts. The overall median survival was eight months with two-year and five-year survival rates of 15% and 3%, respectively. The respective values for patients who had resectable tumors were 21.7 months, 57% and 20%. Ascending cholangitis was a frequent complication in patients treated with dilation and intubation and, overall, hepatic failure and systemic infection were more frequent causes of death than distant metastases.