Congenital posterior nostril atresia is a rare congenital malformation with a prevalence of 1/5000 to 1/8000, unilateral posterior nostril atresia is more common in females than in males. Congenital posterior nostril atresia is a developmental malformation that can be combined with other malformations or syndromes. They can be combined with other malformations or syndromes, such as congenital heart disease and genitourinary malformations. In bilateral atresia, the newborn has difficulty breathing and cannot suckle, which can lead to death by asphyxia or starvation because the newborn cannot open its mouth to breathe. If a newborn presents with dyspnea, cyanosis and asphyxia after birth, and the symptoms disappear or are relieved when crying and worsen when breastfeeding, the disease should be thought of if there is no abnormality in cardiopulmonary examination. The diagnosis can be confirmed by fiberoptic laryngoscopy, and CT films can be used to understand the anatomical abnormalities of the posterior nasal foramen and to distinguish between membranous bony and fused bones. The principle of surgical treatment is to create a wide posterior nasal aperture with a safe and rapid surgical approach and few complications. The treatment is posterior nasal foramenoplasty with postoperative dilatation of the nasal passage with silicone tubes, which relieves respiratory distress and allows normal feeding. Since children with this disease are often seen for respiratory distress, increasing physicians’ awareness of this disease can reduce missed and misdiagnosis. Recently, two cases of congenital posterior nasal atresia (bilateral, bony) were successfully treated in the Department of Otolaryngology of Guangdong Women’s and Children’s Health Hospital under the leadership of Director Zou Yu.