Unilateral pulmonary vein atresia is a rare anomaly, usually associated with symptoms of recurrent hemoptysis and pneumonia in early childhood. Only one report of an asymptomatic adult patient can be found in the literature. We present the case of an asymptomatic patient with unilateral right pulmonary vein atresia in a 20-year-old man. Chest radiograph and multidetector computed tomography showed not only pulmonary vein atresia, pulmonary artery hypoplasia, but also cystic lung changes on thin section CT, along with septal and bronchovascular bundle thickening, and ground-glass opacity. Unilateral pulmonary vein atresia could be another disease which can show cystic lung changes on thin section chest CT.