Extrahepatic portal vein occlusion (EPVO) is an important cause of extrahepatic portal hypertension and variceal hemorrhage in children. We report 3 children with a diagnosis of EPVO and intractable esophageal variceal bleeding who underwent scheduled endoscopic variceal ligation (EVL) and prophylactic propranolol therapy for eradication of esophageal varices. Complete variceal obliteration was achieved in all 3 children following 3-4 treatment sessions. There were no significant complications or gastrointestinal hemorrhage during a follow-up period of 9 to 31 months after variceal eradication. In conclusion, EVL and long-term propranolol prophylaxis is safe and effective in eradicating bleeding esophageal varices in children with EPVO.