Cleidocranial dysostosis is a rare genetic disorder that manifests various abnormalities in bone and dental development. Affected individuals show varying degrees of skeletal, dental, and craniofacial abnormalities. Among its many pathognomonic features, hypoplastic maxilla, depressed midface, mandibular prognathism, and multiple retained deciduous teeth and impacted supernumerary teeth may be treated by collaboration between an oral surgeon and an orthodontist. Herein, we report a successfully treated case of a Korean male adult patient, who was diagnosed with cleidocranial dysostosis, by careful planning and execution of combined orthodontic and surgical treatments consisting of a series of dental extractions of multiple retained deciduous teeth and impacted supernumerary teeth, window opening, button bonding and orthodontic traction of impacted permanent teeth, pre-surgical orthodontic treatment, and orthognathic surgery involving Le Fort I osteotomy, bilateral sagittal split osteotomy, malar augmentation, and partial glossectomy. The treatment resulted in notable esthetic and functional improvements and patient satisfaction.