Aortoarteritis is a chronic inflammatory disease mainly affecting the aorta and its major branches. Recent immunogenetic studies indicate that certain human leucocyte antigen (HLA) alleles are significantly associated with aortoarteritis in several populations. The purpose of the present study was to investigate the relationship between the HLA-DRB1 alleles and aortoarteritis in a Chinese Han population. HLA-DRB1 genotypes were identified by PCR-SSP and PCR-RFLP in 84 Chinese patients with aortoarteritis and 102 healthy Chinese controls. It was found that the HLA-DRB1*04 allele (38.1% in patients vs. 15.7% in controls, p<0.001, relative risk (RR)=2.43) and the HLA-DRB1*07 allele (47.6% vs. 10.8%, p<0.001, RR = 4.42) were significantly associated with aortoarteritis. Furthermore, there was no significant difference in the frequency of the DRB1*0405 subtype between the patient and control groups. Thus the susceptibility to aortoarteritis in this Chinese Han population was closely related with the HLA-DRB1*04 and DRB1*07 alleles. Thus individuals with the HLA-DRB1*04 and DRB1*07 alleles may be at higher risk for developing aortoarteritis.