Carnitine (L-3-hydroxytrimethylamminobutanoate) is a naturally occurring compound that can be synthesized in mammals from the essential amino acids lysine and methionine or ingested through diet. Primary sources of dietary carnitine are red meat and dairy products; however, commercially produced supplements also are available and have been shown to be safe in humans. Carnitine is stored primarily in skeletal muscle, with lower concentrations in plasma. Biologically, carnitine is essential for the transport of long-chain (carbon chain length = 10) fatty acids across the outer- and inner-mitochondrial membranes (carnitine palmitoyltransferanse I and II, respectively). Conflicting results characterized the early research focused on L-carnitine supplementation's ability to enhance endurance performance, and studies showed no changes occurred in muscle carnitine levels. Nevertheless, promising findings for its use have been observed for various pathologies, including cardiovascular diseases, which show it might mitigate some negative effects and enhance physical function. Recent studies have focused upon a different paradigm for L-carnitine in regulating hypoxic stress and enhancing recovery from exercise.