In this study, we investigated the HLA allele and haplotype frequencies, and the association of HLA alleles with serious complications and angiographic findings in Korean patients with Takayasu arteritis (TA) compared with healthy controls. Sixty-one patients (56 women, 5 men), diagnosed with TA between January 1995 and December 2005, were studied. Ninety-five healthy men and women were selected as controls. Clinical manifestations were assessed and angiographies were performed at the time of diagnosis in all TA patients. Genotypes of the HLA-A, -B and -DRB1 loci were determined using the polymerase chain reaction-sequencing-based typing (PCR-SBT) method. The mean age at the time of diagnosis of TA was 37.0+/-12.1 years. Compared with controls, the frequencies of A*3001 (p=0.048), B*5201 (p=0.025), and DRB1*1502 (p=0.046) alleles were significantly higher in TA patients, and the frequency of A*2602 was significantly lower in TA patients when compared with controls (p=0.047). The haplotype containing A*2402-B*5201-DRB1*1502 was significantly increased in TA patients (chi2=5.45, p=0.01). Further, among the serious complication of TA, congestive heart failure (CHF) was found to be associated with B*5201 (OR=5.94, p<0.05, 95% CI=1.04 33.85). These data suggest that A*3001, B*5201, and DRB1*1502 alleles might increase the susceptibility to TA, while A*2602 might protect against TA. Further, our results reveal that the haplotype A*2402-B*5201-DRB1*1502 could be a risk factor for TA, and the allele B*5201 is significantly associated with CHF.