Introduction: The multifactorial etiology of malocclusion involves several factors such as genetic, epigenetic and environmental factors. Among various dental anomalies, the development of supernumerary teeth is also directly related to genetic factors and mesiodens is the most frequent type of anomaly. Case Report: Two monozygotic twin sisters, both of which presented mesiodens, but only one with associated anterior open bite. Supernumerary removal was performed in the two sisters, and the anterior open bite was treated with the use of a fixed palatine grid. Results: The case was finished with proper occlusion and intercuspidation, contemplating facial and dental esthetics. Conclusion: Observing the possible differences that occur in pairs of monozygotic twins, it can be concluded that the development of malocclusions is associated with an interaction between genetic, epigenetic and environmental factors. Thus, the diagnosis and treatment of malocclusion should be individualized for each patient.