Segmental odontomaxillary dysplasia (SOD) is a rare, unilateral developmental disorder of the maxilla involving abnormal growth and maturation of the bone, lack of one or both premolars, altered primary molar structure, delayed tooth eruption, and fibrous hyperplasia of the gingiva. In this, the twenty-third reported case of SOD, the literature is reviewed, and the clinical, radiographic, and histopathologic data are described. Computed tomographic scans of this case showed that the involved segment of the maxilla extends mesiodistally from the permanent cuspid to the mesial portion of the first permanent molar, largely limited to the area of the missing premolars. However, the affected bone extends superiorly in the lateral wall of the maxilla to the zygoma and base of the orbit. This article is intended to serve as baseline data for a future article, describing the natural history and possible treatment of SOD, which remain undocumented.