We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and lower respiratory tract infection. The simple chest X-ray revealed loss of volume, increased reticular interstitial markings of the involved lung and the shift of mediastinal structures to the involved side, and hilar prominency in the contralateral side. Lung perfusion scan revealed no visualization of involved lung. Cardiac Catheterization revealed pulmonary artery hypertension 2/4 and pulmonary artery wedge pressure 1/2. On pulmonary angiogram, nonvisualiaztion of pulmonary vein in the involved side in 3: partial visualization of pulmonary vein not connected to atrium in 1: dysmorphic pulmonary artery on the contralateral side in 1. Right pneumonectomy was done without specific problems in two cases. In conclusion, Unilateral pulmonary vein atresia should be suggested when the clinical and radiological characteristics are present.