We report the patients with extrahepatic biliary atresia seen in the sibships, which is the fourth report in Japan. First patient is a full-term boy with 3,020g in birth weight and the second is a full-term girl with 2,732g in birth weight. Both presented with subdural hematoma and jaundice at the age of 1 month. Pregnancy and delivery were normal and they have no significant history of exposure to infectious disease, illness and drug. A hepatoportojejunostomy was performed at the age of 48 days and 55 days respectively. Both patients had biliary drainage but subsequently had frequent episodes of cholangitis, which led to biliary cirrhosis. Both died at the age of 5 years 6 months and 1 year 4 months respectively. These cases suggest that there might be some kind of inheritance and maternal factor concerned with an etiology of biliary atresia.
