The presence of an uncommon birth defect characterized by an absence of bile ducts from the portal areas in the more peripheral portions of the liver in two male infants of the same family could be mere coincidence, yet the possibility that this anomaly could be hereditary in this family cannot be excluded. Bile duct anomalies such as congenital obliteration of the common bile duct and congenital hepatic fibrosis have already been reported in two or more members of the same family.