Basal cell nevus syndrome (BCNS) is frequently accompanied by multiple maxillomandibular cysts. This disease is known to cause several types of maxillofacial deformity, but there are few reports of orthognathic surgery in patients with BCNS. A patient with BCNS who underwent maxillary distraction osteogenesis is described. The patient was a 19-year-old man. He underwent palatoplasty for cleft palate while he was an infant. Maxillary hypoplasia subsequently developed. Le Fort I osteotomy and anterior alveolar osteotomy of the mandible were performed. A RED system and lateral expansion device were used for maxillary advancement and palatal widening. The total advancement was 15mm, and the total lateral expansion was 6 mm. After distraction, orthodontic treatment was continued. At present, 3 years after distraction, an excellent facial outline and occlusal condition have been obtained. After the operation, we have kept the patient under close observation.
