Central nervous system (CNS) diseases are a group of neurological disorders that affect the structure or function of the brain or spinal cord and seriously threaten the life of patients. A series of researches show mitochondria promotes neuronal development, remodeling, neurotransmitters release and provides enough energy for neurons. Mitochondria dysfunction plays a vital role in the occurrence and development of the CNS diseases. It is also important for timely and effectively improving the function of mitochondria for the CNS diseases. It has been found that exogenous mitochondrial transplantation (EMT) can replace damaged mitochondria, improve and restore cell and tissue dysfunction. EMT, as a powerful therapy for the CNS diseases, will be a great achievement as an important result used for clinical translation in the treatment of CNS diseases. This review aims to describe the origin and development of EMT and further discuss the function and the potential mechanism of using exogenous mitochondria for treatment different CNS diseases.