Behçet's disease is a multisystemic vasculitis of unknown etiology, which is characterized by recurrent urogenital ulceration, cutaneous eruptions, ocular manifestations, arthritis and vasculitis, and its diagnosis is based on clinical criteria. Superior vena cava (SVC) thrombosis is a rare but well-recognized manifestation of Behçet's disease, whereas SVC syndrome due to vasculopathy, without evidence of thrombosis, has not yet been described in the literature. The authors report the case of a patient with Behçet's disease, who presented SVC syndrome with reduction in the lumen of the SVC due to thickening of the vessel wall, without evidence of thrombosis upon computed tomography and magnetic angioresonance. The patient received early anticoagulant therapy, corticosteroid and monthly cyclophosphamide pulse therapy. Clinical control without recurrence was observed after 6 months of follow-up. Behçet's disease should be suspected in young patients presenting with SVC syndrome, in the absence of thrombosis or of a hypercoagulable state.