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Pleuroparenchymal fibroelastosis (PPFE) is a relatively rare interstitial lung disease (ILD) consisting of elastofibrosis involving the subpleural parenchyma and visceral pleura with an upper lobe predominance. It can be idiopathic or associated with some forms of autoimmune disease. The authors describe the case of a 78-year-old woman with a previous diagnosis of Crohn’s disease (CD). She presented with a protracted respiratory infection (with no significant history of previous infections), and underwent high-resolution chest computerized tomography that eventually showed alterations compatible with PPFE. After exclusion of other possible underlying causes, a possible link to CD was considered. Follow-up investigation at 12 months showed clinical and radiological stability and also stable lung function under treatment with hydroxychloroquine and prednisolone. This is the first report of PPFE in the context of CD. Future studies will be important to further investigate this pathological association and its prognostic implications.