OBJECTIVES: The goal was to determine the incidence and types of airway abnormalities in patients with Arnold-Chiari malformation (ACM). METHODS: The study was a retrospective chart review of 24 patients with ACM who were evaluated and treated between November 1991 and August 1997. RESULTS: Eighteen (75%) and 6 (25%) of the 24 patients had types I and II AGM, respectively, Three (12.5% of 24 patients) of the type LI ACM patients had vocal cord impairment: 1 bilateral paralysis, 1 bilateral paresis, and 1 unilateral paralysis. None of the type I ACM patients had vocal cord impairment. Tracheotomy was necessary in 3 of the 24 patients and all in patients with type II ACM. Central sleep apnea was found in 5 of 6 type II ACM patients, but not in any of the type I ACM patients. CONCLUSIONS: Vocal cord impairment and deep apnea were found in 12.5% and 21%, respectively, of this ACM population. When type II ACM patients were considered separately, the incidences of vocal cord impairment and sleep apnea were 50% and 83%, respectively Type II AGM patients tend to have a higher Incidence of airway abnormalities and other neurologic dysfunctions. Flexible fiberoptic laryngoscopy is recommended in the airway evaluation of ACM patients. Early recognition, diagnosis, and management of these abnormalities may be lifesaving.