Background: With the advent of medicine, there is an increasing number of congenital heart disease survivors, which mandates assessment of the long-term risk of stroke and outcomes. We aimed to systematically review the pooled global prevalence of stroke in adults with congenital heart disease (ACHD). Methods: PubMed/Medline, SCOPUS and EMBASE were reviewed until May 2021 to ascertain articles describing stroke rates in ACHD compared to non-ACHD patients over a long follow-up (at least ≥3 years). Random effects models were used to estimate the pooled prevalence of stroke in ACHD and risk of stroke in ACHD vs non ACHD. Heterogeneity was assessed using the I 2 statistics with >75% value suggesting substantial heterogeneity, for which subgroup analysis was performed. Results: We included 14 studies comprising 96,626 participants with ACHD and 751,460 without ACHD. The overall pooled prevalence of stroke in ACHD was 3.7% (95CI 2.5%-5.0%, I 2 = 99%) over a median follow-up period of 11.5 years ( Fig. 1a ). The ACHD patients showed nearly 3 times higher risk of stroke [OR 3.11 (95% CI 2.09-4.63), I 2 =97.99%, p<0.01] on 11.5 years median follow-up ( Fig. 1b ). On subgroup analysis, studies with older ACHD patients showed nearly two times higher rate of stroke compared to studies with younger patients (mean age>40 years: 4.8% vs. mean age ≤40 years, 2.6%). Furthermore, the prevalence of stroke was higher among the ACHD cohorts with shorter median follow-up (<10 years) vs. studies with a median follow-up of >10 years [5.0% vs 3.0%]. Studies published from Denmark (4.5%) and Canada/USA (3.2%) revealed greater rates of stroke in ACHD than the studies from Sweden (2.8%). Conclusion: This meta-analysis found nearly three times higher risk of stroke in ACHD patients over a decade follow-up compared to controls. The demographic, chronological and regional variation of the stroke prevalence in ACHD indicates that the risk could be multifactorial and warrants further risk stratification measures