Mucopolysaccharidosis type IVA (MPS IVA), or Morquio A syndrome, is a rare lysosomal storage disorder caused by a deficiency in the enzyme N-acetyl-galactosamine-6-sulfatase (GALNS). This enzyme deficiency leads to the accumulation of glycosaminoglycans (GAGs), specifically chondroitin-6-sulfate and keratan sulfate, in multiple tissues throughout the body, including bone, cartilage, aorta, heart valves, and the trachea. Patients with Morquio A often present with significant co-morbidities, including respiratory, cardiac, vision, hearing, and skeletal disorders that necessitate repeated medical procedures. Most concerning for the anesthesia provider are the complex airway abnormalities prevalent in these patients. There are three aspects of difficulties with airway management with these patients: (1) GAG deposits throughout the upper airway tissues and the tongue, making ventilation via face mask difficult; (2) difficulty in visualizing larynx and vocal cords when intubating the trachea; and (3) tortuous and narrow trachea rendering passage of an endotracheal tube difficult. Cardiac involvement in Morquio A is common and includes valvular disease, which may progress and necessitate surgical intervention. Another critical aspect of anesthesia care is protecting the spinal cord from ischemia or infarction during surgery, especially during prolonged procedures or in situations complicated by hypotension and hypoxemia. Hence, neuromonitoring in consultation with experienced orthopedic surgeons is recommended. Our skeletal dysplasia team provides comprehensive Delaware consultation for both local and international medical teams managing these complex cases ( https://www.google.com/search?q=skeletal+dysplasia+nemours&rlz=1C1GCEA_enUS1047US1047& ).