A case of Behçet disease which vascular lesions progressed remarkably and had massive gastrointestinal bleeding is reported. A 39 year old man was admitted to the hospital complaining of intractable abdominal pain and angiography revealed impending rupture of a saccular aneurysm just above the bifurcation of the aorta. Aneurysmectomy and patch closure of the defect were performed. Four months later, the patient was admitted again complaining of severe abdominal pain and rerupture of the patch graft was recognized by RI angiography. Aorto-iliac bypass was carried out. Although postoperative course was excellent, the sign of superior vena cava syndrome appeared gradually. Venography revealed occlusion or stenosis of the bilateral subclavian and brachiocephalic veins and the superior vena cava. Femoral venography revealed occlusion of the inferior vena cava, too. Two years and three months later of the first operation, the patient had hematemesis and the extensive varicose vein of the entire esophagus was seen by the upper gastrointestinal examination. Five months later of the examination the patient suddenly had massive gastrointestinal hemorrhage and died in spite of vigorous resuscitative therapy. This esophageal varices was considered as downhill esophageal varices caused by superior vena caval occlusion, not by portal hypertension. Downhill esophageal varices due to mediastinal tumor is seen sporadically, but one due to superior vena caval occlusion secondary to Behçet disease is very rare.