Coenzyme Q10 plays a pivotal role in mitochondrial respiratory chain for cell power supply. Coenzyme Q10 serves as a physiological electron (e–) shuttle from complexes I and II to complex III, as well as a potent antioxidant. In different types of neurological disorders, dysfunction of the respiratory chain leads to reduced ATP levels and increased generation of reactive oxygen species. Coenzyme Q10 supplementation has been widely used in such targeted therapies, including aging, stroke, neuromuscular diseases, Parkinson’s disease, Alzheimer's disease, progressive supranuclear palsy, autosomal recessive cerebellar ataxias, amyotrophic lateral sclerosis and Huntington’s disease. This book summarized a large number of experimental and clinical trials for Coenzyme Q10 based therapies in Neurology.