We prospectively studied 21 consecutive patient with extrahepatic portal venous obstruction for evidence of biliary tract disease. Two patients were firs seen with extrahepatic cholestasis; another had recurrent cholangitis. All three patients with clinical manifest biliary disease were adults. Another five patients had icterus on clinical examination. Live function tests revealed elevated bilirubin levels in 1 patients (66.6%), elevated alkaline phosphatase level in 17 (80.9%) and elevated serum ALT levels in (38.0%). Endoscopic retrograde cholangiography revealed abnormal findings in 17 patients (80.9%). The changes involved the common bile duct (66.6%) more often than they did the hepatic bile ducts (38.1%). Cholangiographic abnormalities included strictures (52.4%), caliber irregularity (23.8%), segmental upstream dilatation (42.8%), ectasia (9.5%), collateral veins causing extraluminal bile duct impressions (14.3%), displacement of ducts (9.5%), angulation of ducts (4.7%) and pruning of intrahepatic ducts (9.5%). The pathogenesis of such cholangiographic abnormalities is unknown. However, possible factors in such changes include collateral veins bridging the blocked portal vein, causing bile duct impressions; fibrous scarring of porta hepatis, causing angulation of bile duct; and ischemic injury to bile duct, leading to stricture formation and caliber irregularity. Biliary disease is important in the clinical outcome of patients with extrahepatic portal venous obstruction because variceal sclerotherapy has prolonged the life expectancies of such patients.