Idiopathic perforation of extrahepatic bile duct is a rare cause of cholestasis in children. A three week's old female newborn presented with cholestasis, irritation, vomitus and abdominal distension on physical examination. The presence of biliary tract cyst was suspected based on abdominal sonography. The laparotomy showed perforation of extrahepatic bile ducts at the junction of the cystic and common bileducts with formation of bile pseudocyst.

Keywords: Cholestasis, bile pseudocyst, children