Serious intestinal bleeding from vascular ectasia secondary to extrahepatic portal thrombosis is much less frequent than variceal bleeding, and its treatment is not clearly defined. We describe a 4-year-old girl with repeated intestinal bleeding from vascular ectasia, without any varix, with late extrahepatic portal vein thrombosis (PVT) and late hepatic artery thrombosis (HAT) after living-related liver transplantation. The bleeding stopped after simple splenectomy. She has presented neither bleeding nor any serious complications related to splenectomy for 1 year to date. We think uncontrollable hemorrhage from gastrointestinal vascular ectasia secondary to extrahepatic portal thrombosis in a pediatric patient can and should be treated by simple splenectomy, because patients with this complication usually have a normally functioning liver. However, it is not clear whether this procedure is effective for variceal bleeding.