1. In the present study, we investigated the effects of the R219K polymorphism of the ATP-binding cassette transporter A1 (ABCA1) gene on serum lipid levels and the response to statin therapy in Chinese patients with coronary heart disease (CHD). 2. The study population consisted of 365 patients with CHD and 246 control subjects without signs or symptoms of CHD. Patients with CHD were treated with 20 mg/day pravastatin. Fasting serum lipids were determined before and after 12 weeks of treatment. Genotyping was performed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). 3. The R219K polymorphism of the ABCA1 gene was not significantly associated with CHD (P > 0.05). Compared with controls, patients with the RR genotype had significantly higher serum triglyceride levels and lower high-density lipoprotein-cholesterol (HDL-C) levels than those with the KK genotype (P < 0.05). In addition, the effects of pravastatin in increasing HDL-C levels were significantly greater in patients with the KK genotype compared with those with the RR genotype (P < 0.05). 4. In conclusion, the R219K polymorphism of ABCA1 was associated with altered lipoprotein levels and the R219K variant significantly modulated the HDL-C response to pravastatin in Chinese patients with CHD.