Background: Pleuroparenchymal fibroelastosis (PPFE) is one of the rare idiopathic interstitial pneumonias (IIP); however, clinical features and prognosis are not well defined in Korean patients. Methods: Eighty two patients, who were diagnosed by lung biopsy (surgical lung biopsy: 52, transbronchial lung biopsy: 32) from 2007 to 2019, were included and clinical data of them were retrospectively reviewed. Conclusions: Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, a tenth of the patients experienced pneumothorax and one-fifth died from pneumonia