BACKGROUND AND PURPOSE: Hepatoblastoma is the most common primary liver cancer in children, yet comprehensive understanding of its epidemiology is limited globally. We aimed to estimate the global trend of hepatoblastoma in children from 1990 to 2021. METHODS: We collected data on hepatoblastoma in children aged 0 to 10 years from 1990 to 2021, derived from Global Burden of Disease (GBD) 2021. Three disease burden indicators, including incidence, mortality, and disability-adjusted life-years (DALYs), were studied. The corresponding average annual percentage changes (AAPCs) were used to explore the temporal trends of hepatoblastoma. RESULTS: In 2021, hepatoblastoma accounted for 4048 incident cases, 2416 deaths, and 213,478 DALYs globally. Incidence, mortality, and DALYs of hepatoblastoma decreased significantly from 1990 to 2021, with AAPCs of -2.12, -2.53, and -2.53. The highest incidence of hepatoblastoma was observed among those aged <
28 days in 2021 (2.57 per 100,000 individuals). Only high-income region showed an upward trend in incidence from 1990 to 2021, with an AAPC of 0.57. The Western Pacific region had the fastest decrease in the incidence, mortality, and DALY rate of hepatoblastoma. Human development level (HDI) was positively associated with the AAPC in incidence from 1990 to 2021, while HDI was negatively associated with the incidence, mortality, and DALY rate of hepatoblastoma in 2021. CONCLUSION: Global efforts over the past 3 decades have substantially decreased the disease burden of hepatoblastoma in children. However, increases in the incidence of hepatoblastoma in high-income region merit attention. The highest disease burden of hepatoblastoma was observed in the neonatal period. Improved understanding of hepatoblastoma epidemiology may facilitate prevention and management.