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Background and objective: The condition characterized as mixed of idiopathic inflammatory arthritis that appears in children younger than 16 years of age has been called Juvenile idiopathic arthritis (JIA). The association between IL-1B gene single nucleotide polymorphism in the promoter region (SNP: -511C/T) with the incidence of JIA in Iraqi children samples depending on genders and ages was investigated in this study. Methods: levels of IL-1B were estimated by ELISA and genetic analysis was done by PCR-RFLP for investigation of SNP: -511C/T in patients and control groups. Results: The results of the present study suggesting highly significant differences in IL-1B levels (pg/ml) between JIA and the control group (p-value< 0.05). The results suggesting allele frequency and genotyping analysis that statistically significant differences by odds ratio and p-value in CC and TT between JIA and control groups but not significant of CT genotypes( OR=1.899, CI 95%=1.34-2.33). IL-1B levels (pg/ml) were investigated in all subtypes of JIA depending on genotypes of IL-1B gene in the patients group and the results were significant in O and S subtypes of JIA (p-value< 0.05). Conclusion: IL-1B SNP:-511C/T appears ahigh-risk factor for physiological incidence of JIA in Iraqi Children.