The authors report the case of a 7-month-old Japanese infant with congenital intrahepatic portosystemic shunts. He had hypergalactosaemia and hyperammonemia at age 1 month. The diagnosis was made by ultrasonography and angiography. Coil embolization was performed successfully, and then hyperammonemia and hypergalactosaemia improved soon after surgery. J Pediatr Sorg 34:1037-1032. Copyright (C) 1999 by W.B. Saunders Company.