Swyer-James-MacLeod syndrome is a rare disease that results in parenchymal damage believed to be caused by recurrent lung infections in childhood. We report a case of a previously healthy young woman that presented with insidious progression of exertional dyspnea with restrictive lung physiology. Axial imaging demonstrated a hyperinflated emphysematous right lung with lower lobe fibrosis, nodules and air-fluid filled cysts, with a small right pulmonary artery and contralateral shifting of the mediastinum. She underwent right pneumonectomy ultimately resulting in improvement of her symptoms, with surgical pathology indicating extensive emphysema, bronchiec- tasis, fibrosis with osseous metaplasia, and placental transmogrification of alveolar septa, being consistent with a diagnosis of SJMS.