A 29-year-old woman with idiopathic portal hypertension was referred to our department for the surgical management of repetitive bleeding from esophageal and gastric varices. At the age of 16 years she had undergone a splenectomy with esophageal transection followed by endoscopic sclerotherapy which had been performed a total of 24 times. Although vericeal hemorrhage was prevented for several months, bleeding from gastric varices and portal hypertensive gastropathy was not able to be controlled readily by endoscopic sclerotherapy from when she was 26 years old. On admission, angiographic studies showed a complete obstruction of the portal vein; however, a portosystemic shunt operation was not able to be performed due to her previous splenectomy. To control her repetitive bleeding, we decided to perform a total gastrectomy and distal esophagectomy with reconstruction by a Roux-en-Y esophagojejunostomy. Her postoperative course was uneventful, and no episodes of recurrent bleeding or other complications have developed, indicating that her quality of life has dramatically improved. Thus, we conclude that distal esophagectomy and total gastrectomy constitute an effective surgical treatment for unshuntable extrahepatic portal hypertension.