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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. DE measured by ultrasonography was higher in the supine position than the standing position. The findings may suggest greater diaphragm movement in the supine position, leading to successful rehabilitation and improved exercise endurance. There is no effective treatment for iPPFE; therefore, an innovative treatment strategy is warranted.