Coenzyme Q10 (CoQ10) plays a pivotal role in mitochondrial respiratory chain which is the cell power supply. CoQ10 serves as a physiological electron (e-) shuttle from complexes I and II to complex III, as well as a potent antioxidant. Neurons are characterized by high rates of metabolic activity and need to respond promptly to activity-dependent fluctuations in bioenergetic demand. Consequently, it is not surprising that mitochondrial alterations can promote neuronal dysfunction and degeneration. In several neurological disorders, dysfunction of the respiratory chain leads to reduced ATP levels and increased generation of reactive oxygen species. CoQ10 supplementation has been widely used to treat aging, stroke, neuromuscular diseases, Parkinson's disease, Alzheimer's disease, progressive supranuclear palsy, autosomal recessive cerebellar ataxias, amyotrophic lateral sclerosis and Huntington's disease. Here we discuss a large number of preclinical and clinical trials fo...