Idebenone (IDBN) is an antioxidant compound, structurally related to coenzyme Q10. It's therapeutic potential is growing in different areas. Over the two decades ago, for potential treatments of mitochondrial diseases have been suggested, relatively few have undergone controlled clinical trials. This review focuses on the recent history of manuscripts of idebenone, coenzyme Q10 and exercise in both primary disorders and secondary disorders. Despite prior clinical impressions that IDBN had a positive effect and significant benefit on Mitochondrial disorders.