This report reviews 164 cases derived from 2 Australian and 3 U.S. centres. There were 128 direct operations and 63 shunts. There were 16 deaths (10% mortality) but 3 were due to unrelated causes. Direct procedures; In the long term these appear of little value. 37 of 48 children having splenectomy subsequently rebled. Almost all children having ligation of varices and direct operations on the stomach rebled subsequently. Acute bleeding can almost always be controlled by conservative measures and direct operations would appear to offer no benefit over non-operative management except in the occasional case of catastrophic management that cannot be controlled conservatively. Shunts; A properly performed decompressive shunt offers the best hope of long term control for bleeding varices. Meso-caval shunts seem to give somewhat better results than splenorenal shunts. About two-thirds of the patients undergoing shunts remain free of any further bleeding. Non-operative management; 27 children have had no surgery performed and all are alive except for one child who subsequently died in a railroad accident. Bleeding episodes become less frequent after the age of 15 years and there are a number of reasons for this including the progressive development of natural shunts. We are not aware of any deaths or major complications from hypersplenism. Growth and development of all children in this series has been normal, although other have commented on a significant incidence of encephalopathy. Conclusions; 1. GIT bleeding becomes progressively less after the age of 15 years. 2. Direct operations have little place in treatment. 3. Decompressive shunts are the most effective method of controlling continuing bleeding but require a shunt of at least 1 cm diameter. 4. Splenomegaly and hypersplenism are not serious problems. 5. One can anticipate the progressive development of natural shunts. 6. Splenectomy should be avoided in this disease unless accompanied by a shunt.