OBJECTIVE: The purpose of our study is to describe the radiologic findings of adult patients presenting with congenital unilateral pulmonary vein atresia. CONCLUSION: Chest radiography in affected patients typically reveals a small hemithorax and ipsilateral pulmonary artery as well as ipsilateral septal thickening. CT shows, in addition, ground-glass attenuation, the absence of a pulmonary vein connection to the left atrium, and abundant mediastinal venous collateral vessels. MR imaging is helpful in further characterizing the vascular abnormalities. Angiography may help to confirm the diagnosis.