This chapter discusses congenital anomalies of the larynx and trachea, where lesions from the nose and mouth to the trachea may be the source of obstruction, with the larynx being the most common origin. Magnetic resonance imaging is the latest diagnostic tool in airway obstruction and has been shown to be an effective method of diagnosing the level of airway obstruction in Down syndrome children. The physiological importance of the cartilaginous support structure is emphasized by the fact that most clinically relevant congenital anomalies of the upper airway involve defects in the cartilaginous subcomponent. The key to securing an airway is to intervene before the situation becomes an emergency. During endoscopic evaluation, the tongue can be pulled forward to see, if the obstructive symptoms are adequately alleviated. Flexible bronchoscopy is superior to rigid bronchoscopy in the evaluation of dynamic tracheomalacia. Interval bronchoscopy is required to confirm normal tracheal growth.