Congenital unilateral pulmonary vein atresia is a very rare anomaly that generally presents during childhood or adolescence as recurrent episodes of pulmonary infections or hemoptysis. Its presentation in adults is highly exceptional. We describe two patients with congenital unilateral pulmonary vein atresia: the first is a 26-year-old woman with recurrent episodes of hemoptysis, and the second is a three-year-old boy with a history of bronchospasm and recurrent pneumonia from the age of three months. Although the diagnosis has classically been reached using cardiac catheterization and pulmonary angiography, we think that the CT and MRI findings can enable the diagnosis of congenital unilateral pulmonary vein atresia in cases with compatible clinical presentation. We emphasize the capacity of MRI for characterizing the vascular anomaly present in these patients.