Segmental odontomaxillary dysplasia (SOD) is a rare craniofacial developmental disorder. Clinical features include abnormal growth and maturation of bone, premolar agenesis, delayed eruption of permanent molars, and unilateral posterior maxillary enlargement. Radiographic features include altered bone trabeculae, reduced maxillary sinus, pulp stones, and spontaneous resorption of primary molars. The purpose of this report is to describe the case of a seven-year-old boy who presented with dental pain, erythema of the soft tissues of the right maxillary quadrant, severely infra-occluded primary molars and bony expansion of the maxilla. Surgical exploration under general anesthesia preceded removal of the infraoccluded primary molars and histopathological examination of atypical alveolar bone. The unerupted teeth were examined, mobilized, and left in situ. Following stabilization, a removable prosthesis was constructed to aid esthetics. A comprehensive approach to treatment is indicated in such cases.