A case of combined valvular disease with the scimitar sign is reported. A 48-year-old woman was admitted to our hospital for treatment of congestive heart failure due to combined valvular disease following an upper respiratory infection. Chest radiography revealed hypoplasia of the right lung, dextroposition of the heart and a curved vascular opacity adjacent to the right cardiac border having the appearance of a scimitar sign. The latter was better demonstrated by chest tomography. A lung scan showed a marked decrease in perfusion of the right lung. A CT scan of the thorax revealed hypoplasia of the right lung, dextroposition of the heart and an abnormal, curved and thickened right pulmonary vein which drained into a markedly enlarged left atrium. Right heart catheterization demonstrated elevated pressures in the right side of the heart. However, no shunt was demonstrated. A pulmonary angiogram revealed an anomalous pulmonary vein, having the appearance of a scimitar sign draining normally into the left atrium. Only five cases like the present case have been reported in the world literatures. The results in the present case indicate that patients with radiological evidence of the scimitar syndrome should be examined using computed tomography of the chest to rule out the "pseudoscimitar sign."