Coenzyme Q10 is an important electron carrier and antioxidant for mitochondrial respiratory chain， and as a synthetic analogue of coenzyme Q10， idebenone was first developed and marketed for clinical use in Japan. Idebenone can act as an antioxidant， transfer electrons， and exert an anti-inflammatory effect， and the human body has better absorption capacity and bioavailability for idebenone. Up to now， a large number of clinical trials have been conducted for idebenone， and preliminary results show that idebenone has the potential to be used in several hereditary and degenerative diseases of the nervous system， such as Leber’s hereditary optic neuropathy， Friedreich ataxia， Duchenne muscular dystrophy， and Alzheimer’s Disease. The article reviews the pharmacological mechanism and several important clinical trials of idebenone， so as to provide a reference for further clinical research and application of idebenone.