Segmental odontomaxillary dysplasia has only recently been delineated from other osseo-odontogenic dysplasias. Yet, this seldom-reported developmental abnormality might be diagnosed by a unique combination of clinical, radiographic, and histologic findings. Unilateral maxillary hyperplasia combined with displaced molars and unusual bone trabeculation is quite obvious at an early age. Recognition and subsequent reporting by dental specialists is encouraged to more clearly define the natural progression and the appropriate treatment of this abnormality. (Am J Orthod Dentofacial Orthop 2003;123:84-6)