Morquio-Brailsford syndrome, mucopolysaccharidosis IV, is a rare congenital hereditary disorder of connective tissue which occurs in severe (type A) and milder (type B) forms. Each has a specific enzymatic defect associated with degradation of keratin sulphate, a constituent of cartilage, which then accumulates and affects bone growth. Dwarfism results, with cardiorespiratory problems and instability of the atlanto-axial joint expected during general anaesthesia.