CXC chemokine receptor type 4 (CXCR4) is a G protein-coupled transmembrane receptor. It is extensively expressed in the body and plays a key role in numerous physiological and pathological processes such as embryonic development, hematopoiesis, cell migration, tumorigenesis and inflammation. These diverse functions give CXCR4 the potential to become an imaging and therapeutic target. In recent years, the radiolabeled CXCR4 ligand 68Ga-pentixafor has been used in non-invasive positron emission tomography (PET) imaging. The technique plays important role in the diagnosis and management of various diseases. This article will elaborate on the clinical application development of 68Ga-pentixafor PET/ CT.