Hunter syndrome (mucopolysaccharidosis, type II; MPS II) is one of a heterogeneous group of recessively inherited mucopolysaccharide storage diseases. Patients with mucopolysaccharidosis show progressive involvement and derangement of many organs, especially upper airway anomalies, which are the major cause of perioperative death. In recent years, a CO2 laser is often applied to upper airway lesions. A 16-year-old patient suffering from Hunter syndrome was scheduled for CO2 laser surgery because of sleep apnea and respiratory stridor. Otolaryngological examination revealed bulging of the bilateral false cord with stenosis of the glottis. We adopted sevoflurane mask induction and high-frequency jet ventilation to overcome the perioperative airway problems. The anesthetic course was uneventful, and the patient was discharged 2 days after the operation.