Behcet's disease is a chronic multisystem vasculitis of undetermined origin. The aorta, vena cava, and pulmonary arteries are affected in approximately 25% of patients. The major diagnostic criteria for Behcet's disease are orogenital ulceration, erythema nodosum, thrombophlebitis and ocular lesions. Pulmonary involvement in Behcet's disease is unusual and it is encountered in only approximately 5% of patients. Recurrent episodes of dyspnea, cough, chest pain, hemoptysis, and fever are cardinal symptoms of pulmonary involvement. Multiple segmental and subsegmental perfusion defects with mismatched ventilation scan findings can show pulmonary involvement of Behcet's disease and pulmonary thromboembolism. Helical computed tomography (CT) can provide better anatomic details of vascular and parenchymal structures and can help to reveal the causes of perfusion defects. The authors describe a patient with Behcet's disease in whom ventilation-perfusion imaging and helical CT findings revealed pulmonary involvement.