Midline segmental odontomaxillary dysplasia.

Segmental odontomaxillary dysplasia (SOD) is a rare developmental disorder of the maxilla, characterized by variability of its clinical and radiological features and may mimic other fibro-osseous lesions. Clinically, the disorder is often diagnosed in early childhood due to a unilateral buccolingual expansion of the posterior alveolar process, gingival enlargement, absence of one or both premolars in the affected region, delayed eruption of the adjacent teeth, and malformations of the primary molars. We describe a rare case of a SOD in a 19-year-old female comprising findings similar to earlier reports, but for the first time SOD is reported along midline. She presented with pre-maxillary enlargement and abnormal pattern of eruption of anterior maxillary permanent teeth. Radiographic imaging showed abnormal bony trabeculation. Histopathologic findings showed characteristic features of SOD. We herein report a case of this rare unusual anomaly and review the literature. Clinicians should be aware of its presence when encountering patients presenting with facial asymmetry unresponsive to treatment.