Pleuroparenchymal fibroelastosis is a rare, progressive fibrotic lung disease characterized by pleural scarring and excessive deposition of elastic fibers, predominantly affecting the upper lobes. Although the etiology remains incompletely understood, PPFE has been reported in association with lung and bone marrow transplantation, chronic hypersensitivity pneumonitis, and certain occupational exposures. Clinically, patients typically present with progressive dyspnea, nonproductive cough, weight loss, and platythorax. The condition is also notable for an increased risk of spontaneous pneumothorax. High-resolution computed tomography commonly reveals pleural thickening along the apical regions of the upper lobes, with subjacent subpleural fibrosis leading to parenchymal contraction and decreased lung volumes. Histologically, PPFE is characterized by marked proliferation and expansion of elastic tissue that infiltrates the subpleural lung parenchyma. Management is primarily supportive, encompassing pulmonary rehabilitation, nutritional optimization, and supplemental oxygen therapy for hypoxemia. Trials of corticosteroids and antifibrotic agents have been reported; however, evidence supporting their efficacy remains limited. The overall prognosis is poor, and lung transplantation remains the only effective treatment option for patients with advanced disease.