Portacaval shunt was successfully employed in the treatment of Type I glycogen storage disease. Severe hypoglycemia, acidosis, hyperlipidemia, and hyperuricemia were corrected and have not recurred although the child is on a regular diet without medication. Based upon our result and the limited experience of others, total portal venous diversion is recommended in early infancy in children with severe hypoglycemia before permanent cerebral damage has occurred.