Abstract: Congenital choanal atresia is a rare life threatening disorder in neonates, (incidence 1 in 5000-7000 live births), but it is the most common indication of surgical intervention of the nose in neonates. It is caused by failure of resorption of the Bucconasal membrane of Hochstetter or Buccopharyngeal membrane of the foregut (ruptures by fifth or sixth weeks to form the choanae) during embryonic development. Generally, 65% to 75% of patients with choanal atresia are unilateral, whereas the rest are bilateral. Bilateral choanal atresia presents with severe respiratory distress and cyanosis at birth and is alleviated by crying whereas unilateral atresia often remains undetected till late in life.

Neonates with bilateral choanal atresia (BCA) require immediate intervention for respiratory distress because they are obligate nasal breathers due to the nasoreceptor reflex. We present this is case report of a full term, healthy newborn baby diagnosed with bilateral choanal atresia. For this rare condition, emergency transnasal endoscopic repair to relieve the life threatening nasal obstruction within 24hrs of birth.