Objective: To investigate the complication rate of tracheotomy in patients with mucopolysacchridoses (MPS) type II (Hunter syndrome). Materials and methods: From 2004 to 2005, seven tracheotomy procedures were performed for the airway management in three patients with MPS type II. The complications for each procedure were analyzed, which included the stomal narrowing, granulation formation, infrastomal tracheal stenosis, and wound infection. Results: All tracheotomies in patients with MPS type II resulted in tracheotomy-related complications, though these procedures secured a safe airway. Infrastomal tracheal stenosis was the most frequent complication (85.7%) and stomal narrowing also occurred frequently (71.4%) after each tracheotomy. These complications caused cannula care to be difficult, with revision frequently required. Conclusion: Of the complications observed after tracheotomy, infrastomal tracheal stenosis and stomal narrowing are frequent in patients with MPS type II. Therefore, tracheotomy procedures should be cautiously applied to the MPS type II patients, and the complications associated with tracheotomy should be discussed with caregivers preoperatively. © 2006 Elsevier Ireland Ltd. All rights reserved.