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Pleuroparenchymal fibroelastosis (PPFE) is an uncommon interstitial lung disease. PPFE is characterized by fibroelastosis involving the pleura and subjacent parenchyma with a predominantly upper lobe distribution. While different possible environmental and familial etiologies have been postulated in the literature, the etiology of this disease remains essentially unclear and most cases are considered as idiopathic. In this report, we describe a rare case of PPFE with multiple lung lobe involvements and prolonged asbestos and silica exposure. Our goal is to investigate the exposure of asbestos and silica and try to identify their possible association with PPFE.