Segmental odontomaxillary dysplasia (SOD) is a characteristic developmental abnormality that demonstrates posterior maxillary enlargement, dental abnormalities, altered bone trabeculation, and possible cutaneous findings. Only 62 cases have been reported in the English-language literature. The authors described 3 newly diagnosed cases of SOD, all found in the left posterior maxilla in adolescent female patients; they reviewed the literature to elucidate this rare entity. Because of the complexity and variety of associated dental and craniofacial features, patients with SOD may seek diagnosis from various dental and medical providers. The signs of SOD are characteristic, yet the condition is largely underrecognized among health care professionals, which may lead to unnecessary treatment.