Abstract
Congenital choanal atresia results from the developmental failure of the nasal cavity to connect posteriorly with the nasopharynx. Although congenital choanal atresia is a well-recognized developmental disorder, it is an uncommon condition with an overall incidence of approximately 1 per 10,000 live births. The authors aim to highlight that in very preterm neonates, early diagnosis of bilateral congenital choanal atresia needs a high index of suspicion to avoid life-threatening events. It is considered as one of the neonatal emergencies, and repair is recommended in the first few weeks of life. The authors report an uncommon presentation of a very preterm neonate born at 31 weeks of gestation, diagnosed to have bilateral congenital choanal atresia with repeated failure of a trial of extubation. A transnasal endoscopic repair was performed in the eighth week of life to relieve the life-threatening nasal obstruction.