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Introduction Congenital heart disease (CHD) imposes a significant health burden due to the associated morbidity and mortality. Therefore, evaluating the temporal trends in the incidence and mortality of CHD across the United Kingdom (UK) is of paramount importance to effectively tackle the associated burden.Methods Data from the Global Burden of Disease database was retrieved to evaluate the temporal trends in the age-standardized incidence rate (ASIR) and the age-standardized mortality rate (ASMR) of CHD across the UK as a whole, and across the 4 different nations (England, Northern Ireland, Wales and Scotland) for the period 1990–2019. The Annual Percent Change (APC) and the Average Annual Percent Change (AAPC) were calculated by using the Joinpoint regression analysis software.Results Over the period 1990–2019, an estimated total of 202,307 congenital heart disease cases with a male predominance of 52.3% were reported across the UK. A statistically significant increase in the ASIR of CHD was noted with an AAPC of 0.61 (95%CI 0.59 to 0.63, p<0.001). Stratification at a national level revealed variations in the trends with England and Scotland witnessing a statistically significant increase in the ASIR with an AAPC of 0.85 and 0.23, respectively, whereas Wales and Northern Ireland witnessed a statistically significant decline in the ASIR with an AAPC of -0.47 and -0.024, respectively. Across England, a contrasting variation in the trends was noted. Greater London, East of England, North West England, South East England, West Midlands, and the Yorkshire and the Humber encountered a statistically significant incline in the ASIR with the most prominent incline noted in the West Midlands (AAPC 2.14, 95%CI 2.12 to 2.17, p<0.001). In contrast, East Midlands, North East England, and South West England encountered a statistically significant decline in the ASIR with the most prominent decline noted in the latter (AAPC -0.55, 95%CI -0.57 to 0.53, p<0.001).Mortality wise, an estimated total of 23,321 CHD-related deaths with a male predominance of 54.3% were reported across the UK during 1990–2019. A statistically significant decline in the ASMR has been noted across the UK (AAPC -3.48, 95%CI -3.70 to -3.18, p<0.001). Further analysis revealed a statistically significant decline in the ASMR across the 4 nations with the most prominent decrease noted in Northern Ireland (AAPC -4.51, 95%CI -5.07 to -4.10, p<0.001). Across England, all regions witnessed a statistically significant decline in the ASMR with the most prominent decline noted in Greater London (AAPC -3.85, 95%CI -3.39 to -2.88, p<0.001).Conclusion Despite a statistically significant increase in the incidence of CHD across the UK and the 4 nations over the span of 3 decades, a statistically significant decline in CHD-related mortality has been observed. These findings mandate prompt recognition to determine the underlying factors leading to the increase in the incidence.