Carnitine, 3-hydroxy-4-trimethylaminobutyrate, a small, water soluble molecule that is essential for mitochondrial fatty acid oxidation, is significantly reduced in hemodialysis patients. Uremia-induced carnitine deficiency, which is magnified by dialysis, is associated with symptoms or clinical problems such as anemia hyporesponsive to erythropoietin, cardiovascular diseases, and muscle weakness. This review examines studies dealing with the different clinical aspects of chronic renal failure patients in which carnitine deficiency may play a role and has also examined the studies, which have evaluated the effect of carnitine deficiency treatment. The reports reviewed in this study, including those more recent from our laboratory, have provided data suggesting that chronic renal failure and particularly hemodialysis patients can benefit from carnitine treatment in particular for renal anemia, insulin sensitivity, and protein catabolism. On the other hand, the heterogeneous clinical response to carnitine therapy in dialysis patients, reported by other studies, and the lack of large-scale randomized trials are the rationale for the reluctance regarding a widespread use of carnitine supplements in dialysis patients. Well-designed randomized clinical trials are therefore required to fully address the potentially important carnitine treatment in dialysis patients.