UNLABELLED: Congenital heart disease (CHD) is a major birth defect characterized by structural and functional heart abnormalities. This study provides updated estimates of CHD prevalence, mortality, and disability using the Global Burden of Disease (GBD) 2021 database. We examined the age-standardized rates of CHD prevalence, mortality, and disability-adjusted life years (DALYs) from 1990 to 2021, stratified by gender, age group, and region. Temporal trends were evaluated using the estimated annual percentage change (EAPC), and associations with the Socio-Demographic Index (SDI) were examined to evaluate the influence of socioeconomic development. From 1990 to 2021, the global age-standardized prevalence rate (ASPR) of CHD remained stable (EAPC: 0.04%, 95% CI: 0.03%-0.05%), while the age-standardized mortality rate (ASMR) (EAPC: -2.38%, 95% CI: -2.44% to -2.32%) and DALYs rate (EAPC: -2.34%, 95% CI: -2.40% to -2.28%) showed significant declines. Among the SDI quintiles, low-SDI regions had the highest ASMR and age-standardized DALYs rates, whereas high-SDI regions demonstrated the highest ASPR. Males experienced higher ASMR and age-standardized DALYs rates than females. Meanwhile, infants under 28 days exhibited the highest prevalence rate (1,539.77 per 100,000 population, 95% UI: 1,309.62 to 1,805.88), mortality rate (760.25 per 100,000 population, 95% UI: 609.51 to 944.66), and DALYs rate (68,489.79 per 100,000 population, 95% UI: 54,902.56 to 85,088.24) in 2021. CONCLUSION: Despite the global decline in CHD-related mortality and DALYs, targeted strategies are crucial for low-SDI regions, males, and infants under 28 days. Improving prenatal screening, maternal nutrition, and pediatric cardiology services can address persistent disparities in CHD outcomes. WHAT IS KNOWN: • Congenital heart disease (CHD), characterized by structural and functional heart abnormalities during embryonic development, is one of the most common type of congenital defects. • While the global age-standardized mortality rate (ASMR) related to CHD declined significantly from 1990 to 2017, it remained notably high in low-SDI and lower-middle-SDI regions in 2017. WHAT IS NEW: • Infants under 28 days of age had the highest prevalence, mortality, and Disability-Adjusted Life Years (DALYs) rates related to CHD. • Males had higher ASMR and age-standardized DALYs rates compared to females.