Portal hypertension in Gaucher disease is unusual; the seventh known patient with this complication is reported. Prior to portacaval shunting in this child, a localized obstruction of the inferior vena cava at the subdiaphragmatic level was demonstrated by caval manometry and inferior vena cavography. At autopsy, centrilobular hepatic fibrosis seemed to be responsible for the portal hypertension. Nodular enlargement of the right and caudate lobes of the liver was the cause of the caval obstruction; elevated caval resistance may have contributed to the portal hypertension and possibly was responsible for failure of a portacaval anastomosis. The value of preoperative inferior vena cavography in addition to arterial portography in children with portal hypertension is stressed.