Pleuroparenchymal fibroelastosis (PPFE) is a rare and recently described interstitial pneumonia. It consists of progressive fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes, with defined and reproducible clinical, radiological and histopathological criteria. No effective treatment has yet been shown to modify the natural course of the disease, which vary greatly in the literature. Several conditions have been associated with PPFE, including connective tissue diseases (CTD). The authors present two cases of female patients with a CTD (rheumatoid arthritis and limited cutaneous systemic sclerosis, respectively) who presented with typical bilateral upper lobe thickening in chest-HRCT. In the first case, diagnosis was based on “definite” radiological and histopathological criteria for PPFE, while in the second case diagnosis was established on clinical grounds after discussion in a multidisciplinary team meeting. The authors present these cases of CTD-associated PPFE in order to raise awareness of this entity among clinicians.