Carnitine is a low-molecular-weight compound obtained from the diet that also is biosynthesized from the essential amino acids lysine and methionine. Carnitine has been identified in a variety of mammalian tissues and has an obligate role in the mitochondrial oxidation of long-chain fatty acids through the action of specialized acyltransferases. Other roles for carnitine include buffering of the acyl coenzyme A (CoA)-CoA ratio, branched-chain amino acid metabolism, removal of excess acyl groups, and peroxisomal fatty acid oxidation. The growing body of evidence about carnitine function has led to increased understanding and identification of disorders associated with altered carnitine metabolism. Disorders of fatty acid oxidation and metabolism typically are associated with primary and secondary forms of carnitine deficiency. These disorders, which include increased lipolysis, increased lipid peroxidation, accumulation of acylcarnitines, and altered membrane permeability, have significant consequences for patients with myocardial diseases and kidney failure. Therapeutic administration of carnitine shows promise in treating selected groups of patients who have altered carnitine homeostasis, resulting in improved cardiac function, increased exercise capacity, reduced muscle cramps, and reduced intradialytic complications.