BACKGROUND The natural history of portal hypertension due to extrahepatic portal venous obstruction (EHPVO) in the presence of a non-cirrhotic liver is not well understood. OBJECTIVE To evaluate the presenting features and the natural history of EHPVO by assessing the bleed patterns prior to definitive management in these patients. METHODS Two hundred and seven consecutive patients presenting with history of variceal bleeding due to EHPVO were studied prospectively. Clinical, hematological and biochemical features on presentation, and pattern and severity of bleeding prior to institution of endoscopic therapy or surgery were recorded. RESULTS Ascites was observed in 16% and was invariably transient. Splenomegaly was present in 82%, with hypersplenism in 22%; however, hypersplenism was symptomatic in only 6% of patients. In 127 patients having more than one episode of bleeding, the frequency of bleeding episodes was 0.94/year and transfusion requirment was 1.47 units/year. No fixed pattern of frequency of variceal bleeding was identified. However, number of bleed episodes and transfusion units requirement correlated with each other. The maximum severity of bleeding was between the ages of 12 and 19 years. CONCLUSIONS Hypersplenism causing symptoms is rare in EHPVO presenting with variceal bleed.