In this article the co-existence of pulmonary emphysema with lung fibrosis of typical pattern and distribution for usual interstitial pneumonia (UIP) was compared with idiopathic pulmonary fibrosis (IPF) alone. Author discusses the etiopathogenesis of these diseases, differences in signaling pathways and the role of senescent cells. Moreover, clinical course, pulmonary function tests as well as main complications are reviewed. However, the lack of well-established diagnostic criteria for CPFE along with mainly retrospective character of the studies make current knowledge about this entity rather deficient.