Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare form of idiopathic interstitial pneumonia. We report a case of a patient with iPPFE in whom postural changes improved diaphragmatic excursion (DE) and exercise tolerance. Chest radiography showed a greater elevation of the diaphragm at maximum expiration in the supine position than the standing position. https://www.kerrenneur.com/product-category/arrow-add-on/