A case of a 47-year-old woman with pulmonary varix is reported herein. Saccular dilatation of the inferior pulmonary vein resembled a pulmonary perihilar mass which could not be palpated at the time of thoracotomy. Aneurysmal dilatation of the pulmonary vein, otherwise known as pulmonary varix, is rare. Only 71 such cases, including 17 cases in Japan, have been reported. Pulmonary varices may be classified into three types, namely: saccular type, tortuous type and confluent type. Most of the varices seen in patients with valvular disease have been of the confluent type (62 per cent), however tortuous type varices have also been seen in some cases (19 per cent). Pulmonary venous hypertension may be one of the major causes of confluent type pulmonary varices as regression of pulmonary varices after mitral valve replacement has been reported. None of the saccular type cases, however, were accompanied by valvular disease. This indicates that local factors may also be an important cause of saccular type varices.