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Background: Mitochondrial diseases are a complex group of pathologies caused by mutations in genes coded in the nuclear and/or the mitochondrial DNA, which fulfill their function in the organelle. The diagnosis is also complex, since it depends on the integration of biochemical, histological and imaging data. Objective: In this paper we review the use of some of these substances and their efficacy in the treatment of these disorders. Conclusions: Currently, there is not a fully effective treatment, however, it was demonstrated that the use of some vitamins, amino acids, antioxidants along with a proper diet and exercise have an important effect that leads to improve the overall state of patients with mitochondrial disorders.