ABSTRACTL‐carnitine plays an essential role in the β‐oxidation of fatty acids by catalyzing their transport into the mitochondrial matrix. The kidney maintains plasma free L‐carnitine levels in the homeostatic range by selective saturable tubular reabsorption. The preferential retention of free L‐carnitine over acyl‐L‐carnitines by the kidney is lost in patients with end‐stage renal disease (ESRD). Loss of renal parenchyma as a site of carnitine synthesis, as well as nonselective clearance of L‐carnitine by the dialysis procedure lead to dialysis‐related carnitine deficiency. Numerous studies investigating whether L‐carnitine supplementation will alleviate several dialysis‐related symptoms, such as intradialytic hypotension, heart failure, muscle weakness, low exercise capacity, and anemia, have reported conflicting results. Many of these studies suffer from a lack of randomization and control groups, heterogeneity in the administration of L‐carnitine, and nonstandardized measures of symptom improvement. More data exist to support the use of L‐carnitine in selected anemic dialysis patients with very large erythropoietin requirements in whom extensive examination for reversible causes of anemia was unrevealing.