Because of the medical characteristics of this inheritable disorder, the treatment of these patients can be fraught with potential pitfalls. Oral manifestations can include taurodontism of the pulp chambers, retrognathia, palatal constriction with attendant posterior crossbite, frontal tongue posturing, and mouth breathing relating to airway restriction from choanal atresia. The authors describe their treatment of a patient three years of age with a tracheostomy.