Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World countries. The etiology and clinical presentation are different in children and adults. The portal vein is transformed into a cavernoma, resulting in portal hypertension and oesophagogastic varices. In addition, extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins resulting in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.