The authors report a rare case of hemangioendothelioma of the right innominate vein complicated by superior vena cava syndrome. Considering development of enlarged venous collaterals, we performed circular resection of superior vena cava together with the right and left innominate veins without replacement. There were no postoperative complications. The use of vascular prosthesis in patients with long-standing superior vena cava compression and enlarged collaterals is associated with high risk of thrombosis and subsequent pulmonary embolism. Safe circular resection of superior vena cava without replacement is possible after intraoperative test clamping.