Behcet's disease is a multisystemic disorder that the main pathological defect is vasculitis and superior vena cava syndrome (SVCS) caused by vasculopathy is a rare manifestation of the disease. Although successful results of endovascular surgery were observed in some cases, medical therapy may have priority among the treatment alternatives because of the high risk of surgery due to the lesional localization of the vessel. In this report, a Behcet's disease case with SVCS and 18 months' follow-up data with medical therapy is presented.