Background/Aims: Carcinoma of the proximal bile ducts is a rare condition that occurs more frequently in men than in women. The majority of patients present with obstructive jaundice and the main aim of treatment should be to relieve jaundice while at the same time, if possible, removing the tumor. However, even when surgical resection is contraindicated or technically impossible, it should still be possible to achieve satisfactory palliation of jaundice. Methods: We ana]yzed the 72 patients with carcinoma of proximal bile ducts who were admitted to Pusan National University Hospital from January 1982 to December 1993. Results: The mean age of the patients was 69.1 years old and the ratio of male to female was 1.88:1. The clinical manifestations showed jaundice(97%), right upper quadrant pain(31%), pruritus(22%), epigastric pain(19%) and weight loss(117c). Prevalence rate of proximal cholangiocarcinoma according to the level of lesion in proximal bile duct was 71% in Klatskin tumor(Bismuths type I 16%, type II 0%, type III 6%, type IV 49%), 11% in common hepatic duct, 9% in common bile duct(the area in which cystic duct is communicated with common hepatic duct in this article), and 9% in intrahepatic duct (left 3%, right 6%). The Clonorchis sinensis infestation was associated in 50%, which was the most common associated disease with proximal cholangiocarcinoma. The cholangitis was associated in 5k, the sepsis in 15%, the hepatitis B in 117o, and the liver cirrhosis in 10%. The choledocholithiasis was associated in 5%, which consists of the gallstones in 3%, the common bile duct stones in 1%, and intrahepatic duct stones in 1%. The direct invasion of surrounding structures and metastasis were found in 23 cases. Among 23 cases, direct invasion was found in 18 cases, especially liver invasion in 14 cases, and distant metastasis in 4 cases, among which peritoneal metastasis was 2 cases. Staging could be reviewed in 70 cases. Among them, stage I was found in 46 cases(69%), stage III in 3 cases(3%), stage IV in 21 cases(28%), and stage II was not found. Percutaneous transhe- patic biliary drainage(PTBD) was performed in 43 cases (60%) and T-tube insertion in 7 cases (10%). Nothing was performed in 22 cases(30%). Conclusions: A clinical awareness of the manifestation of proximal cholangiocarcinoma will result in early diagnosis and treatment. Even when surgical resection can not be performed, palliation of jaundice should be required for prolonged survival of patients. (Korean J Gastroenterol 1995;27:700-710)