Objective: To evaluate whether individuals with osteogenesis imperfecta (OI) are more affected by malocclusion than individuals without OI.
Materials and Methods: Searches in PubMed, Ovid, Web of Science, Scopus, Lilacs and gray literature were performed. Data extraction was conducted by two researchers. Risk of bias assessment employing the Newcastle-Ottawa Scale and meta-analysis were conducted. Results were provided with mean difference (MD), odds ratio (OR) and 95% confidence interval (CI). Strength of evidence was determined. Results: Six cross-sectional studies were included. In comparison with individuals without OI, the group with OI had 19.69-fold greater chance of exhibiting Angle Class III malocclusion (OR = 19.69, CI: 9.00-43.09) and presenting anterior crossbite greater (MD = 6.08, CI: 2.40-9.77). Individuals without OI had a significantly greater ANB angle (MD = 3.88, CI: 1.15-6.61) and SNA angle (MD = 2.11, CI: 0.24-3.98) in comparison with those with OI. No difference between groups was found for SNB (MD = −0.50, CI: −2.21 to 1.21) and open bite (MD = 0.98, CI: −0.29 to 2.25). Most studies included had moderate methodological quality. Strength of evidence was low or very low. Conclusions: The occurrence of Angle Class III malocclusion and anterior crossbite was greater among individuals with OI compared to those without OI. These findings can assist stakeholders about the occlusal abnormalities affecting OI individuals.