Coenzyme Q10 (CoQ10) plays a critical role in mitochondrial oxidative phosphorylation by serving as an electron carrier in the respiratory electron transport chain. CoQ10 also functions as a lipid-soluble antioxidant by protecting lipids, proteins and DNA damaged by oxidative stress. CoQ10 deficiency has been associated with a number of human diseases including mitochondrial diseases, neurodegenerative disorders, cardiovascular diseases, diabetes, cancer, and with the ageing process. In many of these conditions CoQ10 supplementation therapy has been effective in slowing or reversing pathological changes. Oxidative stress is a major contributory factor in the process of retinal degeneration. In this brief review, we summarize the functions of CoQ10 and highlight its use in the treatment of age-related macular degeneration and glaucoma. In light of these data we propose that CoQ10 could have therapeutic potential for other retinal diseases.