Congenital airway malformations encompass a broad array of disorders that occur at diverse anatomic levels. The presentation of these disorders varies widely and is influenced by the level at which obstruction occurs as well as the severity of the obstruction. In view of the distinct anatomy of the pediatric airway and the possibility of airway symptoms rapidly progressing to life-threatening airway compromise, early detection, diagnosis, and appropriate management are essential. The aim of this chapter is to present an overview of congenital anomalies extending from the larynx to the distal airway, briefly describing patient assessment, symptomatology, and contemporary management strategies.