Statins lower cholesterol by inhibiting 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, the rate-limiting enzyme in the biosynthesis of cholesterol. However, severe adverse events, including myalgias and rhabdomyolysis, have been reported with statin treatment. Different mechanisms have been proposed to explain statin-induced myopathy, including reduction of mevalonate pathway products, induction of apoptosis, mitochondrial dysfunction, and genetic predisposition. A decrease in coenzyme Q(10) (CoQ), a product of the mevalonate pathway, could contribute to statin induced myopathy. This article reviews the clinical and biochemical features of statin-induced myopathy, the inter-relationship between statins and the concentration of CoQ in plasma and tissues, and whether there is a role for supplementation with CoQ to attenuate statin-induced myopathy.