A case of an association of extrahepatic biliary atresia (EHBA) and urogenital sinus (UGS) anomaly that was diagnosed as an urachal remnant antenatally is reported. Diagnostic laparoscopy of the abdominal cavity in the postnatal period was the most helpful step for making the diagnosis. Biliary atresia and urogenital sinus, which was the cause of bladder outlet obstruction, were treated successfully. To the authors' knowledge, this is the first case of this type of association introduced to the literature. Copyright (C) 2001 by W.B. Saunders Company.