The treatment of esophageal variceal hemorrhage is still the subject of some controversy. The main causes of portal hypertension in children are portal vein thrombosis or cirrhosis, most commonly caused by biliary atresia. Many treatment options are available including endoscopic, radiographic, and surgical strategies. In general, children with presinusoidal obstructions have preserved hepatic synthetic function, and, therefore, treatment options include endoscopic strategies or portosystemic shunts, each with advocates. For children with advanced liver disease, liver transplantation offers the only chance for cure, so primary treatment of variceal bleeding should be by endoscopic means or transjugular intrahepatic portosystemic shunt (TIPS). Each modality has specific advantages and disadvantages, and treatment recommendations must therefore be tailored to the individual on a case-by-case basis, largely dependent on the expertise and experience of the health care team.