Choanal atresia or stenosis is the most common craniofacial defect of the nose. In choanal atresia there is either partial or complete obstruction of the one or both openings (posterior choanae) connecting the nasal cavity to the pharynx. Bilateral choanal atresia presents at birth with life-threatening airway obstruction, as neonates are obligate nasal breathers. The etiology of choanal atresia is largely unknown. Infants with bilateral choanal atresia will require stabilization of their airway soon after birth. Surgical repair should be performed early in bilateral choanal atresia if there are no medical contraindications.