OBJECTIVES: The purpose of this study is to determine if there is a survival advantage to having a tracheostomy in patients with tracheal cartilaginous sleeve (TCS), to determine if the age of the patient at the time of tracheostomy affects morbidity, and to determine if patients with a true pars membranacea have a survival advantage and less morbidity than those without a pars membranacea. STUDY DESIGN: Case report and meta-analysis of the literature were conducted. METHODS: A review of the world literature from 1979 to 2006 was performed. All reports of patients with craniofacial syndromes found to have TCS by autopsy or endoscopic findings were included. A case report of a new patient with TCS is presented. RESULTS: Patients who undergo tracheostomy have a statistically significant survival advantage (P = .0067). The patient's age at the time of tracheostomy was not associated with survival time (P = .45). There is no association with absence or presence of a pars membranacea and clinical symptoms of respiratory distress (P = .50). There is no overall difference in survival between patients with a pars membranacea and those without (P = .78). CONCLUSION: Tracheostomy placement in patients with TCS and craniosynostosis can decrease morbidity and increase survival. Interval bronchoscopy is important to treat tracheal stoma granulation tissue. There is no survival advantage to having TCS with a pars membranacea.