Isolated pulmonary vein atresia without associated congenital malformation is a very rare condition in adults. It is associated with significant mortality and morbidity. Recurrent pulmonary infections, dyspnea on exertion and hemoptysis may be the presenting complaints. Owing to the limited number of patients with this condition there may be some difficulties in it's diagnosis and therapy. We report an adult case with this rare congenital malformation and discuss it's diagnosis and management options.