Twelve patients with portal hypertension caused by biliary cirrhosis were treated from January, 1983- June. 1990. There were 6 women and 6 men. Age ranged from 20 to 50 years with an average of 35.5 years. In all patients, a significant feature of this series was the long period of biliary obstruction caused by bile duct stones (N = 7), congenital atresia of biliary tract (N = 1), Caroli disease (N = 1), sclerosing cholangitis (N = 1), sclerosing carcinoma of biliary tract (N = 1), and benign postoperative stricture of biliary tract (N = 1). The incidence was 6.6% in portal hypertension; 2.2% in biliary tract disease and 18% in intrahepatic cholelithiasis. Esophagogastric varices were demonstrated in 6 cases. In 5 cases with or without esophageal varices, varices around the extrahepatic bile duct were detected before or during operation. Some complications including poor liver function, dilated collaterals around extrahepatic bile duct and biliary infection with underlying biliary stones existed simultaneously, therefore the management of these cases was very difficult. There was no single approach to patients with portal hypertension caused by biliary cirrhosis and it should be treated as an individual basis.