A 14 year-old adolescent with achondroplasia and Jeune's syndrome (asphyxiating thoracic dystrophy) presented for cervical spine surgery in the prone position. Due to the need for home mechanical ventilation at night, the patient had a tracheostomy in place. With the first surgical procedure, the cuffed tracheostomy tube was left in place during prone positioning. Difficulties encountered with ventilation through the cuffed tracheostomy tube in the prone position necessitated aborting the case. During three subsequent surgeries, the tracheostomy tube was removed and an armored endotracheal tube was placed through the tracheostomy stoma prior to prone positioning. No further difficulties with ventilation were noted with the subsequent cases. There are currently no guidelines in the medical literature regarding perioperative management of patients with a tracheostomy requiring prone positioning for surgery. The management of such patients is reviewed and possible problems with tracheostomy positioning during prone positioning are explored. Given such issues, we would suggest removal of the tracheostomy tube and placement of an armored endotracheal tube through the stoma during surgical procedures in the prone position.