Objective To analyze the factors influencing the occurrence of congenital heart disease (CHD) in children in Aheqi County,Xinjiang Uygur Autonomous Region (Xinjiang). Methods 60 cases of children diagnosed with CHD in our outpatient department of Aheqi County People's Hospital from April 2023 to May 2024 were selected as the case group,and 60 healthy children undergoing routine health check-ups during the same period were selected as the control group. The clinical data of the two groups were analyzed,and univariate and multivariate logistic regression was carried out to analyze the influencing factors in the occurrence of CHD in children. Results The results of the univariate analysis showed that the pre-pregnancy BMI≥25.0 kg/m2,living area,family history of CHD,iron deficiency anemia,fever during pregnancy,gestational diabetes,premature birth,vitamin D+calcium supplementation and folic acid supplementation were statistical differences between the two groups in (P<0.05);The results of the multivariate logistic regression analysis showed that,pre-pregnancy BMI≥25.0 kg/m2 (OR:10.816,95%CI:5.394~21.688),living area (OR:6.593,95%CI:2.992~14.525),family history of CHD (OR:3.568,95%CI:1.548~8.223),fever during pregnancy (OR:25.154,95%CI:12.132~52.149),gestational diabetes mellitus (OR:5.430,95%CI:1.866~15.803) were an independent risk factor for developing CHD in children,and supplement of vitamin D+calcium (OR:0.484,95%CI:0.363~0.645),folic acid supplementation (OR:0.346,95%CI:0.228~0.523) were protective factors affecting the CHD,statistically significant for comparison (P<0.05). Conclusion Pre-pregnancy BMI ≥25.0 kg/m2,residence,family history of CHD,fever during pregnancy and gestational diabetes are independent risk factors for the occurrence of CHD in children in Aheqi County,Xinjiang. Regular supplementation of vitamin D,calcium and folic acid are independent protective factors. In order to reduce the incidence of CHD in children,it is necessary to guide pregnant women to adopt more appropriate perinatal management.