Choanal atresia is a rare congenital malformation which occurs in every 5000 to 8000 live newborns. Clinically, it may present as an isolated form or in association with other malformations. In most cases, the cause is not found, but some are associated with a genetic mutation or the use of certain drugs against hyperthyroidism during pregnancy. The dominant clinical picture at birth is respiratory distress with severe cyclic cyanosis. The paper presents the case of a girl with isolated bilateral choanal atresia who showed signs of respiratory distress and cyanosis at birth. The development of the nose and nasal cavity, the etiology, clinical picture, diagnostics, primary care, and surgical treatment of choanal atresia are discussed.