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Coenzyme Q10 (CoQ10) or ubiquinone is the essential coenzyme for cellular aerobic respiration which is the most efficient process in high energy compound generation. The majority of CoQ10 in human body comes from endogenous synthesis. There is only a little amount of CoQ10 from exogenous sources such as dietary. The endogenous biosynthesis of CoQ10 decreases proportionally to the age of the cell or the tissue, thus, individuals lacking CoQ10 and patients with inherited CoQ10 deficiency need an exogenous supplementation to maintain the physiological requirement. Apart from playing a key role in aerobic respiration, CoQ10 also acts as a potential antioxidant. It prevents cellular oxidative stress, which causes several deteriorations related with age. For this reason, CoQ10 has been continuously studied due to its high potential for adjunct therapy in some diseases. It can be used as a supplement, risk prevention and substitution for individuals having a defect of endogenous synthesis. Several studies have reported randomized control trials study suggesting that CoQ10 holds a promising potency for medical application in the near future.