Objective To explore the correlation between cysteyl leukotriene receptor 1 (recombinant cysteinyl leukotriene receptor 1, CysLTR1) gene polymorphism and montelukast treatment responsiveness in children with allergic rhinitis (AR).Methods A total of 310 children with AR treated in our hospital from Jan 2018 to Apr 2021 we prospectively selected. All children received montelukast treatment. According to the treatment response, they were divided into good response group and poor response group. Their baseline data, general clinical data, laboratory data, lung function indicators were collected. Polymerase chain reaction (PCR) was adopted to detect CysLTR1(927T/C、434T/C) gene polymorphism, and Logistic regression analysis to evaluate the relationship between CysLTR1 polymorphism and the montelukast treatment response in AR.Results After treatment, 265 cases (85.48%) had good response and 45 (14.52%) had poor response. The differences in general data such as age, gender, family history between the two groups were statistically insignificant (all P>0.05). The differences in body mass index, proportion of combined asthma, serum 25-hydroxyvitamin D3[25-hydroxyvitamin D3, 25(OH)D3], and CysLTR1 434 T/C polymorphism between the two groups were statistically significant (all P<0.05). Logistic regression analysis showed that the body mass index (OR=1.520, 95%CI=1.045~2.211), combined asthma (OR=97.737, 95%CI=10.656~896.442), CysLTR1 434 T/C polymorphism [CT type (OR=62.486, 95%CI=6.440~606.304), CC type (OR=159.229, 95%CI=15.457~1 640.252)] were risk factors for montelukast treatment response, and serum 25(OH)D3(OR=0.673, 95%CI=0.568~0.797) was a protective factor for montelukast treatment response (all P<0.05).Conclusion CysLTR1 gene polymorphism is associated with montelukast treatment responsiveness in children with AR.