Choanal atresia (CA) is an uncommon clinical entity with an estimated incidence of 1:5000–7000 births. Interestingly, the characteristics of CA follow a “2–1” rule, namely the ratio of unilateral to bilateral CA, female to male, and right-sided to left-sided CA. Multiple congenital anomalies are commonly encountered among these patients, with CHARGE syndrome accounting approximately for 25% of the cases. In case of bilateral CA, infants can have acute respiratory distress with intermittent cyanosis characteristically relieved by crying. Feeding difficulty can be the initial alerting event in which the infants can present with progressive airway obstruction and choking during feeding because of their inability to breathe and feed simultaneously. Infants with unilateral CA, rarely present with neonatal respiratory distress, are often diagnosed later on in life. The most common presentation is chronic unilateral nasal obstruction, persistent mucoid rhinorrhea, and/or a history of chronic sinusitis. Occasionally, the correct diagnosis could not be reached until adulthood. Given the relatively low morbidity of unilateral CA, its definitive treatment is usually delayed until later age. Five main CA surgical approaches have been described: trans-palatal, trans-antral, transseptal, sublabial, and trans-nasal. The trans-nasal endoscopic approach is currently the most frequently used.