The differential diagnosis of coexistent pleural and parenchymal fibrosis includes asbestosis, connective tissue disease associated lung disease, advanced fibrotic sarcoidosis, or drug-induced lung disease. Also, intraalveolar fibroelastosis is not unique to PPFE, and differential diagnosis includes radiotherapy-related changes, drug-induced lung disease, connective tissue disease, certain inhalational injuries, and apical cap. The predominance of elastic fibers in PPFE aids in distinguishing it from other conditions. Given its subpleural distribution, PPFE may be confused with UIP (either IPF or secondary to known causes); however, PPFE is temporally homogeneous, has few fibroblastic foci, and honeycomb change is absent. Also UIP tends to be lower lobe predominant at least initially. Correlation with clinical, radiologic, and serologic parameters is needed for differentiating PPFE from all the other conditions.