Background. Accumulation of glycosaminoglycans is known to cause significant problems in the anesthetic management of children with mucopolysaccharidoses (MPS). Clinical and standard radiological evaluation may convey insufficient information about the upper airway and trachea in children with MPS. Multidetector computed tomography (MDCT) images have been used to define the central airway and previous studies have recommended this tool to assess the airway of children who are considered at risk of difficult intubation. However, MDCT has not been recommended in MPS children. The aim of this clinical scenario study was to verify whether information from MDCT reconstruction of the airway is useful in airway management planning of children with MPS.