ABSTRACT: Molar-Incisor Hypomineralization (MIH) is a qualitative defect of enamel of unknown etiology, affecting one or more permanent molars and may include incisors. This condition is a clinical challenge and its prevalence is still uncertain given the recent increase in research. Thus, we aimed to comprehensively estimate the overall prevalence of MIH and associated characteristics. This systematic review is reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). We searched articles using PubMed, MEDLINE, CENTRAL, Web of Science, SciELO, LILACS and TRIP databases, until July 2021. Heterogeneity and publication bias were computed via I2 test statistics and Egger’s significance test, respectively. Random-effects meta-analysis of prevalence were processed. We used the Strength of Recommendation Taxonomy [SORT] to grading the strength of evidence. Overall, 116 observational studies were included, with one study with moderate methodological quality and the remaining of high methodological quality. Subgroup analysis confirmed an influence of not using the 2003 MIH case definition (p = 0.0066). The pooled prevalence of MIH was 13.5% (95% CI 12.0–15.1, I2 = 98.0%). Affected incisors were seen in 36.6% (95% CI 30.0–43.7, I2 = 92.5%) of the cases. Lastly, the prevalence of hypomineralization of the second primary molars was observed in 3.6% of the MIH cases (95% CI 1.9–6.8, I2 = 96.3%). America was the continent with highest prevalence (15.3, 95% CI 12.8–18.3, p < 0.001, I2 = 96.3%) and Asia had the lowest prevalence (10.7, 95% CI 8.5–13.5, p < 0.001, I2 = 98.7%), however no continental differences were found. Sample size and year of publication were slight contributing factors to the heterogeneity in the analysis. Overall, these results were classified with a SORT A recommendation.