From 1960 to 1981, 31 children, 18 boys and 13 girls, have been treated at Ste-Justine Hospital for extra-hepatic portal hypertension. Age at the onset of gastro-intestinal bleeding 3 1/2 months and 13 years. Splenomegaly with hypersplenism, hematemesis and melena have been the most frequent clinical manifestations. Percutaneous splenoportography be coelio-mesenteric arteriography confirmed the diagnosis of cavernomatous transformation of the portal vein. Among the 31 children, 28 bled from their varices, the 3 others did not to date. Among the 28 patients with active bleeding complication, 7 have been treated conservatively, 11 had sclerotherapy (sclerosing injections of varices). On the surgical point of view, 7 had ligation of varices with intra-thoracic transposition of the spleen in 5 of them. Portosystemic shunts were performed in 10 patients, 3 central spleno-renal and 7 cavo-mesenteric shunt. Each of these 28 patients had an average of 9,5 episodes of gastro-intestinal bleeding, 8.7 hospitalizations, and received 7.5 liter of blood. Medical treatment, sclerotherapy, ligature of varices and intra-thoracic transposition of the spleen are palliative measures. Nevertheless the procedures are time and life savers, allowing improvement and development of natural porto-systemic shunts. Follow up of unshunted patients on a period of 8 to 17 years revealed a decreasing frequency and intensity of the hemorrhagic manifestations. Operative risks and morbidity of recurrent bleeding should be the guidelines for surgery. Natural course of illness and possible neuro-psychiatric consequences of a porto-caval shunt should also be considered.