Proteus syndrome is a rare disorder of undefined etiology. It is characterized by an overgrowth of various tissues, hyperostosis and epidermal and connective tissue nevi. Because of the variety of clinical manifestations, with distribution in mosaic pattern, the diagnosis is difficult and requires a multidisciplinary team to provide adequate professional care. This paper reports the case of a teenager with proteus syndrome presenting severe mandibular retrusion and apnea–hypopnea associated. After creating a multidisciplinary treatment plan, it was decided for the tracheostomy as an immediate alternative. Orthodontic treatment and maxillary disjunction were proposed to initiate treatment of the malocclusion and to prepare for the orthognathic surgery. After this procedure a significant improvement occurred on the patient’s clinical condition, but not sufficient for tracheotomy tube removal. In discussion with the neurosurgery, it was taken into consideration the possibility of anterior osteoplasty of cervical vertebrae, because they showed hyperostosis, occurring posterior compression of the oro pharynx, but the procedure was suspended due to high risk of intraoperative complications. It was proposed, then, a new bimaxillary advancement in order to enlarge the passage of air through the pharynx giving a better quality of life to the patient with the removal of the tracheotomy tube. This patient has been followed for almost eight years.

Conflict of interest: None declared.