Idebenone is an organic compound belonging to the quinone family, being similar to coenzyme Q-10. It is a kind of drug developed by Takeda Pharmaceutical Company for the treatment of Alzheimer’s disease and some other cognitive defects. However, these have been not very much progress associated with this indication. It is now also used for the treatment of Friedreich’s ataxia with a positive effect on cardiac hypertrophy and neurological function. However, this indication is only approved in Canada, not in Europe and US. It is now under investigation on its efficacy for the treatment of Duchenne muscular dystrophy, Leber’s hereditary optic neuropathy, mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) as well as primary progressive multiple sclerosis. The efficacy of this drug still demands more evidences.