Segmental odontomaxillary dysplasia is an uncommon maxillofacial deformity. Clinically, overgrowth and maturation of the maxillary right or left posterior alveolus and the respective gingiva, lack of premolars, delayed eruption of the adjacent teeth, and malformed primary molars are observed. The purpose of this report was to describe the clinical and radiographic findings of a 47-year-old female with segmental odontomaxillary dysplasia and summarize the prosthetic rehabilitation with fixed and removable partial dentures. A 7-year follow-up did not reveal biomechanical or functional problems except the loss of the mandibular left central incisor and wear of the artificial teeth.
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