Vitamins are essential in metabolic reactions in the body as catalysts in enzymatic reactions or as coenzymes carrying chemical groups between enzymes. Many vitamins function in enzyme complexes participating in mitochondrial respiration and energy production or they are required for synthesis of mitochondrial respiratory chain components. Biosynthesis of the component of mitochondrial respiratory chain, coenzyme Q, is dependent on vitamins B 2 , B 6 , B 12 , folic acid, pantothenic acid, niacinamide and vitamin C. Vitamins B 1 , B 2 , B 6 , niacin, biotin, folic acid and pantothenic acid are important for metabolic pathways in mitochondrial respiration and energy production. Vitamins C, E, niacin and folic acid belong to effective scavengers of free radicals, prevent mitochondrial oxidants formation and mitochondrial aging. Some mitochondrial diseases are linked to vitamin deficiencies and can be improved by vitamin supplementation.