Keywords: airway; anomalies; congenital anomalies and syndromes; difficult EN airway anomalies congenital anomalies and syndromes difficult 207 208 2 04/07/20 20200301 NES 200301 Airway management continues to be a leading cause of morbidity and mortality in children. However, noncraniofacial anomalies in the respiratory system, gastrointestinal system, and body habitus can also contribute to the difficult airway management in patients with genetic syndromes such as mucopolysaccharidoses and glycogen storage diseases. Pediatric patients may change over time, and the surgical procedure itself may affect the airway anatomy; hence, presentation in future visits may not be the same and airway assessment need to be comprehensive and individualized at each encounter with the patient.