Congenital unilateral pulmonary vein atresia is a very rare cardiac malformation. In this case, the patient was brought to the hospital for recurrent upper respiratory infection without exertional dyspnea or hemoptysis, and cardiac murmurs were heard on physical examination. The volume of affected lung was lost on simple chest x-ray. Lung perfusion scan revealed no visualization of affected lung. Cardiac catheterization revealed mild pulmonary artery hypertension. Pulmonary angiography showed a small right pulmonary artery, stasis of contrast material and venous phase revealed nonvisualization of draining right pulmonary veins. In this case, the patient did not undergo any surgical treatment for no symptoms other than recurrent upper respiratory infection were reported. We report a case of congenital right pulmonary vein atresia.