Mucopolysaccharidosis is characterized by the progressive accumulation of glycosaminoglycans in multiple organs. Valve and coronary involvement, upper airway obstructive disease, joint stiffness, and mental retardation are associated perioperative anesthetic risks. Nineteen patients and 23 anesthetic cases were presented for elective surgery. The mean patient age was 10.8 years. General anesthesia was administered in 21 cases and intubation was failed in two. Mask ventilation without intubation was performed in two cases in day surgery unit. In one case, spinal anesthesia was performed. Otolaryngologic procedures, i.e., tonsillectomy and adenoidectomy, and ventilation tube insertion were most common. Percutaneous endoscopic gastrostomy and herniorrhaphy were also frequent. Dexamethasone was given to all intubated cases and all patients were extubated in the postanesthesia care unit or in the intensive care unit. There was no perioperative mortality. Cautious airway management until intubation is recommended and mask ventilation with short-acting inhalation or intravenous anesthetics is enough to manage relatively short procedures. For herniorrhaphy, a spinal block could be used.