Behçet syndrome in association with pulmonary abnormalities is rare. We described a patient with Behçet syndrome who had pulmonary involvement. A review of the literature disclosed 12 similar cases. Thrombophlebitis, superior vena cava obstruction, and hemoptysis are characteristic clinical features of patients with Behçet syndrome and pulmonary infiltrates. Once diffuse pulmonary infiltration and massive hemoptysis appear, the prognosis is poor; death from pulmonary exsanguination is likely to occur. Treatment with cyclophosphamide and prednisone, in addition to blood transfusions, may be beneficial.