This report describes a 49-year-old woman with recurrent massive gastrointenstinal bleeding from jejunal varices without portal hypertension. Preoperative diagnosis was obtained by abdominal computed tomography, superior mesenteric arterial angiography and percutaneous transhepatic portography. Percutaneous transhepatic portographic findings revealed no step-up of oxygen concentration and normal pressure in the portal vein, only dilation of superior mesenteric vein. Jejunal resection and anastomosis resulted in complete resolution of the bleeding, and the patient has experienced no recurrent bleeding over a 4 year follow-up period. A review of the literature shows that this syndrome is nearly always characterized by portal hypertension, generally due to liver cirrhosis. Accurate preoperative diagnosis is often difficult. We report a very rare case of jejunal varices without portal hypertension.