Abstract: Objective To investigate the association between multiple system atrophy (MSA) and serum lipids. Methods On hundred and thirteen patients with MSA admitted to Chinese PLA General Hospital from April 2012 to April 2014, and anothe 132 age- and gender-matched healthy subjects were enrolled in this study. Patients’ lipid contents, including total cholesterol (TC) triglyceride (TG), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), apolipoprotein A-(ApoA1), apolipoproteins B (ApoB), were observed. The risk factors of MSA patients were analyzed using logistic regressio analysis. Results Serum lipids like TC, TG, LDL-C and ApoB were signifcantly lower in MSA patients than in controls (TC: 4.3 mmol/L vs 4.70 mmol/L, P=0.003; TG: 1.17 mmol/L vs 1.42 mmol/L, P=0.006; LDL-C: 2.66 mmol/L vs 2.94 mmol/L, P=0.013 ApoA1: 1.17 mmol/L vs 1.28 mmol/L, P=0.006; ApoB: 0.86 mmol/L vs 0.92 mmol/L, P=0.021). While, the levels of TG an apoB showed no signifcant differences between MSA-c patients and controls (P＞ 0.05), and HDL-C level showed no signifcan differences between MSA-c, MSA-p and controls. The multivariate analysis showed that decreased level of TC, LDL-C, ApoA1 an ApoB were risk factors of MSA. Conclusion The levels of TC, TG, LDL-C, ApoA1 and ApoB is lower in MSA than in controls and the decreased level of TC, LDL-c, ApoA1 and ApoB are the risk factors of MSA.