Pemphigus Vulgaris (PV) is a rare, autoimmune bullous disease of the skin and mucous membranes. Corticosteroids are usually the main core treatment for control of PV, which could result in complicating disorders. Objective: This study aimed to determine the lipid-lowering effects of L-carnitine supplementation in PV patients. Methods: In this randomized, double-blind, placebo-controlled clinical trial, 52 patients with PV were divided randomly into two groups to receive 2 g L-carnitine (n=26) or placebo (n=26) for 8 weeks. Serum levels of L-carnitine (LC) and lipid profiles were evaluated at the beginning and end of the study. Results: only 47 participants [LC group (n=24) and placebo group (n=23)] completed the trial. L-carnitine intake considerably decreased serum levels of triglycerides (TG, P=0.02), total cholesterol (TC, P<0.001), low density lipoprotein-cholesterol (LDL-C, P=0.01), and very low density lipoprotein-cholesterol (VLDL-C, P=0.02). In addition, there was a significant increase in serum carnitine (P<0.001) and non-significant change in high density lipoprotein-cholesterol (HDL-C, P=0.06) in comparison with the placebo. Within-group comparisons also demonstrated that serum TG (P=0.001), TC (0.001), LDL-C (P=0.003) and VLDL-C (P=0.001) significantly declined and the mean concentration of carnitine (P<0.001) and HDL-C (P<0.001) significantly increased following L-carnitine supplementation. During the study, no harmful effects were reported by patients.Conclusion: This is the novel study to determine the favorable effects of L-carnitine supplementation on lipid profiles in PV patients. However, further investigations are required to confirm these results.