This study is aimed at investigating the correlations of PCSK9 and LDLR gene polymorphisms as well as serum PCSK9 levels with atherosclerosis and lipid metabolism in patients on maintenance hemodialysis. SNP at the E670G locus of the PCSK9 gene and the rs688 locus of the LDLR gene was analyzed by polymerase chain reaction‐restriction fragment length polymorphism. All study subjects’ blood lipid (triglyceride (TG), total cholesterol (TC), high density lipoprotein cholesterol (HDL‐C), and low density lipoprotein cholesterol (LDL‐C)) concentrations and lipoprotein (a) and PCSK9 levels were measured. The differences in blood lipid levels between different genotypes of the E670G locus of the PCSK9 gene and the rs688 locus of the LDLR gene in maintenance hemodialysis patients with atherosclerosis were compared. Maintenance hemodialysis patients with atherosclerosis at the E670G locus of the PCSK9 gene AG + GG genotype had higher levels of TG, TC, LDL‐C, and lipoprotein(a) than the AA genotype, and lower levels of HDL‐C than the AA genotype. Maintenance hemodialysis patients with atherosclerosis at the rs688 locus of the LDLR gene CT + TT genotype had higher levels of TG, TC, LDL‐C, and lipoprotein(a) than the CC genotype, and lower levels of HDL‐C than the CC genotype. Serum PCSK9 contents in maintenance hemodialysis patients with atherosclerosis were positively correlated with lipid indices (TG, TC, LDL‐C, and lipoprotein(a)) and carotid ultrasound indices (intima‐media thickness and resistance index), and negatively correlated with HDL‐C, maximum systolic blood flow velocity and minimum diastolic blood flow velocity (all P < 0.05).This article is protected by copyright. All rights reserved