While there have been major advances in both the identification of the molecular basis and our understanding of mitochondrial pathology, the clinical management of patients with mitochondrial respiratory chain disease is still essentially supportive. Quinones are the only pharmacological agents that have proven some efficacy when, and only when, given to patients presenting with quite specific respiratory chain defects. In this article, after a short presentation of the coenzyme Q(10) molecule, its origin and distribution in human body, we summarize our present knowledge on its several physiological functions. We next discuss the rational that justifies using different types of quinones in the therapy of mitochondrial disorders. We finally briefly review the available data obtained in the therapy of mitochondrial disorders by using quinones as either substitutive electron carriers or antioxidant compounds.