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The present review was aimed to describe the prevalence and the regional distribution of malocclusion among preschool children worldwide. Two independent reviewers performed a systematic literature search to identify English publications from January 2010 to May 2024 using PubMed, ISI Web of Science and Embase. Search MeSH key words were "malocclusion", "primary dentition" and "child, preschool". The reporting quality was assessed by the modified Newcastle–Ottawa Quality Assessment Scale. We identified 2599 publications and recruited 47 articles. Fourteen of the included studies were conducted in Asia, four in Europe, twenty-eight in South America and one in Africa. The prevalence of malocclusion ranged from 28.4% to 83.9%, and half of the reported prevalences were higher than 50%. The highest percentage was in Asia (61.81%), followed by Europe (61.50%), South America (52.69%) and Africa (32.50%). Statistically significant differences existed in deep overbite, anterior open bite, posterior crossbite, edge-to-edge incisor relationship and distal step between continents (p < 0.05). Europe showed the highest prevalence (33.08%) of deep overbite. Africa showed the highest prevalence (18.60%) of anterior open bite. Europe showed the highest prevalence (15.38%) of posterior crossbite. The most common malocclusion traits were increased overjet and deep overbite. To conclude, malocclusion remained prevalent in the primary dentition and varied between countries. [ABSTRACT FROM AUTHOR]