Swyer-James syndrome (SJS) is a rare disease probably resulting from bronchiolitis obliterans. The radiological findings of this entity are characterized by hyperlucent appearance of one or more lobes of a unilateral lung, decreased lung volume, diminished ipsilateral hilar shadow and hardly visible arterial structure on chest radiography. We report a 50-year-old patient, who had unilateral right lower lung hyperlucency on chest radiography. However, the patient reported a history of pulmonary atelectasis of lobus centralis dexter in the course and the corresponding affliction of ipsilateral pulmonary vein, that rendered the diagnosis more complicated. The radiological and pathological features of this syndrome, as well as differential diagnosis were also discussed.