ObjectiveTo analyze the trend of disease burden changes in congenital birth defects in China from 1990 to 2019. MethodsUsing the global burden of disease study 2019 (GBD 2019), we analyzed the morbidity, mortality, and disability-adjusted life years (DALYs) of congenital birth defect diseases and their corresponding age-standardized rates and average annual percentage change (AAPC) to analyze the changes in the disease burden of congenital birth defects in China and compared them with global data from 1990 to 2019. ResultsIn 2019, the age-standardized incidence, mortality, and DALY in China were 147.41/100 000, 4.62/100 000, 480.95/100 000, respectively. Compared with 1990, the age-standardized incidence rate, age-standardized mortality rate, and age-standardized DALY rate increased by 12.08% and decreased by 70.38% and 66.82%, respectively. In recent years, although the age-standardized incidence of congenital birth defect disease in China is on the rise and higher than the global level, the disease burden is roughly on the decline and lower than the global level, which is closely related to earlier intervention and treatment of the disease resulting in a lower standardized mortality rate. ConclusionThe age-standardized mortality rate of children with congenital birth defects in China showed a decreasing trend from 1990 to 2019, and the burden of disease ranged from slightly higher than global to lower than global levels, but the age-standardized incidence rate was significantly higher, but the age-specific incidence rate has increased significantly.