Non-cirrhotic portal hypertension (NCPH) is a rare cause of bleeding oesophageal varices. The prognosis for patients with NCPH is generally better than that of patients suffering from cirrhotic portal hypertension. Gastrointestinal bleeding or asymptomatic splenomegaly is the usual clinical presentation. If surgery becomes necessary splenectomy alone is therapeutically insufficient. Definitive reduction of portal pressure by complete or incomplete shunts provide the lowest rate of recurrent bleeding and an excellent long-term prognosis. Exact diagnosis of the aetiology of NCPH is of great importance in the correct choice of operative procedure.