Three infants are described in whom acquired biliary atresia developed during the perinatal period. In two cases this was related to a spontaneous perforation of the bile duct, and in the other it probably was related to previous surgery for duodenal and ileal atresias. Clinically, the symptoms in these patients differed from the congenital forms of biliary atresia; two of the infants had dilated intrahepatic ducts on ultrasonography, and all had restriction of disease to the extrahepatic bile ducts and an excellent response to surgery.