Objective: To study the association of the ITPKC gene SNP rs28493229 with Kawasaki disease(KD)susceptibility and formation of coronary artery lesion(CAL).Methods: This study include KD patients group(n=30)and normal children(n=25)as control group.Polymerase chain reaction(PCR)and gene sequence analysis were applied to detect the single nucleotide polymorphism of ITPKC gene rs 28493229.All KD patients were divided CAL group(n=13)and no coronary artery lesion(NCAL)group(n=17).Results: The rs 28493229 locus were detected in all the study subjects,including three genetypes(GG,GC and CC).About this SNP,there were significant differences between KD group and healthy group,KD children with CAL group and NCAL group about genotype frequencies and allele frequencies(P<0.05).C allele increased risk for KD susceptibility and at same time increased risk for CAL in KD children(OR=5.22,95%CI 1.56~17.44; OR=5.50,95%CI 2.88~18.77).Conclusions: Polymorphisms of ITPKC gene SNP rs 28493229 probably influence susceptibility to KD and CAL formation in Han children of north China.