Abstract: Objective To analyze the current situation and trend of birth defects in Jieshou City, and to explore effective intervention measures to reduce the occurrence of birth defects in this area.Methods The data of perinatal reports from 28 weeks of gestation to 7 days of postpartum delivery in two monitoring project sites of national birth defect hospitals in Jieshou City from 2014 to 2018 were analyzed.Results A total of 39124 perinatal infants were monitored. 357 cases of birth defects were reported. The incidence of perinatal birth defects was 9.12 per thousand, showing an upward trend year by year (P<0.05). The top five perinatal birth defects were finger (toe), external ear deformity, congenital heart disease, cleft lip and palate, and brain deformity (including hydrocephalus, encephalocele and anencephalus). The incidence of birth defects was higher in male perinatal infants and pregnant women aged less than or equal to 20 years old or more than or equal to 35 years old.Conclusion The incidence of perinatal birth defects in Jieshou City is increasing year by year. Three-level prevention should be promoted to reduce the incidence of perinatal birth defects.