Pulmonary fibrosis (PF) is a common, persistent, irreversible, fatal chronic lung disease with a median survival of 2-4 years after diagnosis. It is characterized by excessive extracellular matrix deposition and scarring in the lungs, leading to functional failure, severe respiratory problems and even death. Numerous studies have shown that CXC chemokines and their receptors play important roles in PF and other desmoplastic disorders. Several studies have shown that chemokines may become new targets for the treatment of many diseases. Here, we review the role of key CXC chemokines and their receptors in PF to provide a reference for the treatment of PF.