Portal hypertension in the absence of liver disease in children remains a therapeutic challenge. Despite successful control of variceal bleeding in most children, the risk of massive GI bleeding and mechanical disturbance of a huge spleen associated with hypersplenism exists throughout life. Surgical shunt between the portal and systemic venous systems is considered a definitive solution for that problem.We present our experience with the porto-systemic shunt for extrahepatic portal hypertension.Nine children (mean age 11.4 years) with portal hypertension were referred for a shunt procedure during a 5-year period (1996-2001). We reviewed patients charts for clinical parameters before and after surgery as well as surgical data (type of shunt, portal pressure gradient). A mesocaval shunt was constructed in 5 children, a splenorenal shunt in 3 other children and one child with splenic vein thrombosis underwent splenectomy alone for presumed diagnosis of left sided portal hypertension.During a mean follow-up period of 21.2 months (range 6-63 months), the surgical shunt remained patent in 7 of the 8 children. An immediate drop in portal pressure and increased platelets count over time was noted in those 7 children. In two children high portal pressure persisted after surgery, including a child who underwent mesocaval shunt following a previous failed splenorenal shunt and another child who underwent splenectomy alone. None of the children bled following the shunt procedure and decreased splenic size was observed in the 3 children with massive splenomegaly.We suggest that the porto-systemic shunt is indicated for children with non-hepatic portal hypertension suffering with uncontrolled bleeding and those with massive splenomegaly associated with hypersplenism.