Coenzyme Q10 (CoQ10, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain(electron transport chain) with antioxidant properties. In view of the involvement of CoQ10 inoxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone wouldbe expected to contribute to disease pathophysiology by causing a failure in energy metabolismand antioxidant status. Indeed, a deficit in CoQ10 status has been determined in a number ofneuromuscular and neurodegenerative disorders. Primary disorders of CoQ10 biosynthesis arepotentially treatable conditions and therefore a high degree of clinical awareness about thiscondition is essential. A secondary loss of CoQ10 status following HMG-CoA reductase inhibitor(statins) treatment has been implicated in the pathophysiology of the myotoxicity associated withthis pharmacotherapy. CoQ10 and its analogue, idebenone, have been widely used in the treatment ofneurodegenerative and neuromuscular disorders. These compounds could potentially play a rolein the treatment of mitochondrial disorders, Parkinson’s disease, Huntington’s disease, amyotrophiclateral sclerosis, Friedreich’s ataxia, and other conditions which have been linked to mitochondrialdysfunction. This article reviews the physiological roles of CoQ10, as well as the rationale and therole in clinical practice of CoQ10 supplementation in different neurological diseases, from primaryCoQ10 deficiency to neurodegenerative disorders. These will help in future for treatment ofpatients suffering from neurodegenerative disease.