A 26-year-old woman with a history of recurrent spontaneous abortion was admitted because of superior vena cava syndrome. Chest computed tomography and digital subtraction angiography revealed obstruction of the superior vena cava by a thrombus. After admission she also developed oral aphthous ulceration, erythema nodosum, and positive cutaneous hypersensitivity reaction to intradermal injection of saline. Laboratory investigations revealed HLA-B51 and positive lupus anticoagulant. We made the diagnosis of Vasculo-Behçet's disease and treated her with heparin and urokinase with no clinical improvement. The fever subsided after prednisolone administration. Two years later, superior vena cava syndrome had slightly improved, although RI venography showed complete obstruction of the superior vena cava with development of collateral circulation. Endoscopic examination showed downhill esophageal varices. It is of note that the initial symptom in this case of Vasculo-Behçet's disease was superior vena cava syndrome, and that lupus anticoagulant may have contributed to the thrombus formation.