Swyer-James syndrome, a rare disease with unilateral hyperlucent lung due to bronchiolitis obliterans and pulmonary artery hypoplasia, generally develops after lower respiratory tract infection during early childhood. Invasive procedures, including bronchoscopy and angiography, are often necessary for a definitive diagnosis. We report a 17-year-old man admitted because of cystic bronchiectasis complicated by lung abscess. Chest roentgenography showed the typical findings of Swyer-James syndrome. Noninvasive magnetic resonance angiography was used to confirm hypoplasia of the right pulmonary artery. The patient received antibiotic therapy, underwent a right lower lobectomy for the lung abscess, and recovered.