Objective: To identify the association of occlusal disorders in patients with sickle cell disease (SCD). Study design: A literature review was conducted, and articles published between 2010 and 2019 were searched on Bireme and PubMed websites and in MEDLINE and LILACS databases, in English, Portuguese, and Spanish, using the keywords “malocclusion,” “sickle cell disease,” and “cephalometry,” combined by Boolean operators AND and OR. One of the criteria for the selection of articles was the presence of adolescents in the sample. This methodology followed the PRISMA recommendations. Seventy-nine articles were found, seven of which were included in the review as they met the inclusion criteria and the study goals. Results: The prevalence of malocclusion in SCD patients ranged from 62.9% to 100%, which was considered very severe in 30.1% to 80.6%. The most common occlusal changes were Angle’s class II malocclusion, increased maxillary overjet, and anterior open bite. In addition, class II skeletal pattern was the most prevalent due to mandibular retrusion. Conclusion: Malocclusion prevalence in SCD patients is high and considered to be a risk factor, with a significant rate of very severe malocclusion when compared to healthy patients.

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