Demyelinating diseases are a group of nervous system disorders characterized by myelin sheath damage. Demyelinating diseases can seriously affect the quality of life of its victims and still lack satisfying therapeutic options. Oligodendrocyte precursor cells (OPCs) are progenitor cells exist in the central nervous system (CNS), with migration and proliferation capacities and potential to differentiate into oligodendrocytes (OLs), which are myelinated cells in the CNS, indicating that OPCs are closely related to myelination and post-injury regeneration in CNS. Recently, with the improved understanding of the mechanisms of OPCs development and lineage specification, the approaches to gain functional OPCs through directed differentiation from pluripotent stem cells or lineage reprogramming from somatic cells have been greatly promoted. Based on these achievements, OPCs transplantation becomes a promising therapeutic option for the treatment of demyelinating diseases of CNS. In this review, we summarized the latest research progress in this field.