The Swyer-James syndrome is a relatively uncommon disease entity presented with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. The main finding is a hyperlucent lung with small hilar shadows on the chest X-ray. Pulmonary angiography is the standard method for diagnosis. We report a case of the Swyer-James syndrome with a brief review of literature.