Levocarnitine is a molecule required in mammalian energy metabolism. It removes the potentially toxic acyl groups from the cell helping to maintain normal metabolic functions. In addition, it facilitates the transport of long-chain fatty acids across the mitochondrial membrane for beta oxidation and subsequent energy production in skeletal muscle and myocardium. It has been shown in numerous studies that levocarnitine metabolism is abnormal in patients with end-stage renal disease. Significant dialytic loss of levocarnitine has been reported in addition to dietary changes undertaken in this population, which may decrease dietary levocarnitine intake. Recent studies have shown that levocarnitine administration to hemodialysis patients has improved exercise performance, intradialytic muscle cramps and hypotension episodes, and overall well-being. Ongoing and future studies will help to formulate more definite recommendations on the dose and the duration of levocarnitine therapy in dialysis patients.