Background: Carnitine deficiency is a common condition in patients on maintenance hemodialysis that contributes to dyslipidemia, anemia, and poor general health in these patients. We evaluated the effect of oral L-carnitine supplementation on lipid profile, anemia, and quality of life in hemodialysis patients.Methods: This randomized, double-blinded, placebo-controlled, trial was conducted on dyslipidemic patients on maintenance hemodialysis. Patients in the intervention group (n = 24) received 1 g/d of L-carnitine and the placebo group (27 patients) were similarly taking placebo for 16 weeks. Concentrations of plasma triglycerides, total cholesterol, HDL (High density lipoprotein), LDL (Low density lipoprotein), hemoglobin, erythropoietin dose, and quality of life (QOL or Quality of life, using Short-Form Health Survey) were measured at baseline and 8 and 16 weeks after the beginning of the intervention.Finding: After the intervention, there was a significant decrease in triglyceride (-31.1 ± 38.7 mg/dl, P = 0.001) and a significant increase in HDL (3.7 ± 2.8 mg/dl, P < 0.001) and hemoglobin (0.7 ± 1.7 g/dl, P = 0.037) concentrations in the carnitine groop. Decrease in total cholesterol concentration in the carnitine group was not statistically significant (-6.6 ± 16.0 mg/dl, P = 0.055). No significant change was seen in LDL concentration in any group (P > 0.05). Erythropoietin dose was significantly decreased in both the carnitine (-4750 ± 5772 mg, P = 0.001) and the placebo (-2000 ± 4296 mg, P = 0.033) groups. No improvement was observed in QOL scores or its domains in any group.  Conclusion: In patients on maintenance hemodialysis, oral L-carnitine supplementation has significant beneficial effects on lipid profile. Also, it can increase hemoglobin concentration and subsequently reduce needed erythropoietin dose, but has no considerable effect on quality of life in hemodialysis patients.