Hepatoportal sclerosis is the term used to name a clinicopathological condition responsible for non-chirrotic portal hypertension. A three cases report of children with hepatoportal sclerosis is presented associated with portal vein thrombosis. The first two patients presented as main complaint upper digestive hemorrhage and the third one was admitted for investigation of hepatosplenomegaly. The ultrasonographic exam revealed alterations indicative of extrahepatic portal vein thrombosis in the three cases. The patients underwent liver biopsy for they had presented altered liver enzymes. The main histological findings were: subintimal sclerosis, portal fibrosis and telangiectases of the intrahepatic venous branches, consistent with the diagnosis of hepatoportal sclerosis. The three patients showed good evolution, being the hemorrhage controlled in the first two cases through esclerotherapy of esophageal varices.34121-5