Objectives: Patients receiving hemodialysis have been shown to be carnitine deficient due to many causes. Tissues, especially the skeletal muscle and myocardium, require carnitine for the production of energy. This study was performed to find out the effects of L-carnitine supplementation on muscular symptoms and cardiac functions in dialysis patients. Methods: Among 72 hemodialysis patients, 40 patients who showed decreased free carnitine levels were selected to receive L-carnitine intravenously after each hemodialysis session for 6 months. Before and after supplementation, echocardiography, various neurologic examinations and questionnaires were obtained. Results: After carnitine treatment for 6 months (1∼1.5 g per every hemodialysis session), the blood level of carnitine was increased more than 10 times (19.04±7.12 μmol/L vs. 267.24±69.94 μmol/L, P<0.001). The left ventricular ejection fraction was improved in the patients who have less than 60% of ejection fraction (56.45±2.53% vs. 60.44±6.29%, P=0.03) after carnitine treatment. The neurological symptom score and isometric muscle power (pinch power) were improved, but the total neuropathy score, activities of daily living scale and grip power were not changed after carnitine supplementation on dialysis patients. Conclusion: Regular L-carnitine supplementation on hemodialysis patients can improve their left ventricular ejection fraction and some parts of functionality. (Ewha Med J 2012;35(2):89-94)