This study aimed to assess the potential association between perception malocclusion and school performance in children and adolescents. An electronic search was performed in ten databases. Based on the PECO acronym (Population, Exposition, Comparator, and Outcome), the eligibility criteria included observational studies that compared the school performance of children and adolescents with and without the perception of malocclusion. There were no restrictions on the language or year of publication. Two reviewers selected the studies, extracted the data, and assessed the risk of bias by using the Joanna Briggs Institute tool for cross-sectional studies. School performance was measured by analyzing student grades; levels of absenteeism; and child or adolescent self-perception and/or the perception of parents, guardians, close friends, and teachers regarding the impact of malocclusion on school performance. The data were described narratively/descriptively. The search resulted in 3,581 registers, of which eight were included in the qualitative synthesis. These studies were published between 2007 and 2021. Two studies concluded that there was no significant association between school performance and perception of malocclusion, five studies found that only some of the children with malocclusion had their school performance affected, and one study concluded that there was a significant association between perception of malocclusion and low school performance. Considering all variables and the very low certainty of evidence, the perception of malocclusion seems to negatively impact school performance when associated with external and subjective factors. Further studies using additional measurement standards are required.