1. A case of congenital defect of the extrahepatic bile ducts is described. Soon after birth the baby showed some characteristic manifestations of the obliteration of the bile duct such as jaundice, enlargement of the liver, hemorrhagic diathesis, discolored grayish feces and deeply colored urine with bilirubin but no urobilins, and died four months after birth. 2. Autopsy revealed the defect of the extrahepatic hepatic ducts and the common bile duct. No communication was proved between the intrahepatic bile ducts and the duodenum. The gall bladder was found to be attached to the duodenum by a slender and atrophic cystic duct, but not communicated with the intrahepatic bile ducts. 3. Concerning this malformation a discussion and a brief review of literatures are made on the mechanism of development, classification of types and the chief symptoms.