Abstract: Objective　To investigate the association of two single nucleotide polymorphisms (SNPs) (rs2736340 and rs2618476) in BLK gene with Kawasaki disease (KD) and coronary artery lesion in Chinese Han children. Methods　In the case-control study, 179 children with KD were selected and 182 healthy check-up children during the same period were selected as normal controls. The genotypes of two SNPs in BLK gene were detected using PCR-RFLP and the data were analyzed. Results 　There was no difference in distribution of three genotypes (TT, CT, and CC) of SNP rs2736340 between KD group and control group (P=0.093). However, T allele frequency in KD group was significantly higher than that in control group (P=0.021). The distribution of three genotypes (CC, CT, and TT) of SNP rs2618476 between KD group and control group was significantly different (P=0.021). C allele frequency in KD group was significantly higher than that in control group (P=0.006). The two SNPs in BLK gene were not associated with rash, hand-foot edema and coronary artery lesion (CAL), but SNP (rs2618476) was associated with oral mucosa lesions (P=0.018). Conclusions　The SNP (rs2736340) in BLK gene was not associated with KD, but the T-allele was associated with KD. The SNP (rs2618476) was associated with KD in Han Chinese, and was also associated with oral mucosa lesions in KD patients.