Objective: To explore the prevalence and change trend of severe multiple disabling birth defects in Chongqing City from 2007 to 2020, and to provide a basis for comprehensive prevention and control measures of severe multiple disabling birth defects. Methods: Based on hospital monitoring data, 1 103 children with severe multiple disabling birth defects monitored by all birth defects monitoring institutions in Chongqing City from January 2007 to December 2020 were included in the study. They were grouped by year, perinatal gender, maternal permanent residence (urban/rural), maternal age, different regions and other categories. Chi-square test was used to analyze the differences in the incidence of severe multiple disabling birth defects in different categories, and linear trend test was used to analyze the change trend. Results: From 2007 to 2020, a total of 1 022 314 perinatal births and 1 103 severe multiple disabling birth defects were monitored in Chongqing City, with an incidence of 10.79/10 000, including 11.47/10 000 in urban areas and 9.48/10 000 in rural areas, with an incidence higher in urban areas than in rural areas(χ2=8.424,P=0.004). Male was 10.47/10 000, female was 10.97/10 000, there was no significant difference between male and female(χ2=0.606,P=0.436). The incidence of maternal in different age groups was u-shaped, and there was statistical difference in incidence among different age groups (χ2=59.465, P<0.001). The incidence of maternal in<20 years old and ≥35 years old was higher, and in 25-29 years old was lower. The incidence rate was 17.96/10 000 in 2007 and 7.18/10 000 in 2020, which decreased by 60.02% in 2007-2020. The incidence of neural tube defect, chromosome syndrome, limb shortening and abdominal wall defect decreased by 78.12%, 77.38%, 80.55% and 91.30%, respectively. Severe Congenital heart disease rose by 456.16%. From 2007 to 2020, the overall incidence of the disease showed a decreasing trend (χ2trend =117.046, P<0.001), except for the rise of severe congenital heart disease, (χ2trend=8.744, P=0.003), the other four types of diseases were on the decline (neural tube defects χ2trend =48.618, P<0.001; chromosome syndrome χ2trend=50.999, P<0.001; limb shortening χ2trend=73.464, P<0.001; abdominal wall defect χ2trend=79.863, P<0.001). Conclusion: From 2007 to 2020, the incidence of severe disabling birth defects in Chongqing City showed a downward trend, and there were regional and age differences. The incidence of severe congenital heart disease is on the rise.