Introduction: Hurler syndrome (Mucopolysaccharidosis-I) is a lysosomal storage disorder due to deficiency of α-L-iduronidase enzyme. Anaesthetic management in these patients is challenging due to possible upper airway obstruction, difficult intubation, thick secretions, post-obstruction pulmonary oedema, cardiac and cervical spine abnormalities.

Case: Ten year old female child with hurler syndrome underwent keratoplasty in left eye. She had mild OSAS (AHI = 7), trivial mitral regurgitation, kyphoscoliosis and decreased range of motion of multiple joints. She had normal mouth opening, large tongue, short neck and Modified Mallampati Grade 4 view. Anaesthesia was induced with intravenous propofol and Air-Q intubating laryngeal airway of size 1.5 was placed; later changed to size 2 because of leak. Fibreoptic evaluation of airway was done through Air-Q. Intraoperative duration was uneventful. Postoperatively, after awake removal of Air-Q, patient had copious secretions and developed laryngospasm, which was treated with succinylcholine. Patient was mask ventilated with an oropharyngeal airway till she regained adequate spontaneous breathing.

Conclusion: Airway complications are frequent in mucopolysaccharidoses and laryngeal airways are useful in the management. Air-Q offers the added advantage of easier intubation through it when compared to other supraglottic airways. It has been shown to provide a better fibreoptic view of glottis. Air-Q can be used to safely secure the airway in patients with Hurler syndrome.