Piro’s sequence syndrome is a disorder characterized by a small jaw deformity, posterior tongue drop and airway obstruction, with a characteristic U-shaped incomplete cleft palate in approximately 58-90% of children. Piro’s sequence does not constitute a separate syndrome, but occurs alone or as a manifestation and reflection of other syndromes to varying degrees. The airway obstruction caused by the small jaw deformity and posterior tongue drop makes it difficult for the child to breathe and eat, which in turn leads to hypoxemia, gastroesophageal reflux, severe malnutrition and, in severe cases, progressive weight loss, wasting and even death. The treatment of Piro’s sequence is a multidisciplinary and comprehensive one. The type and location of airway obstruction and the causes of feeding difficulties are evaluated before treatment measures are taken. The treatment plan is designed to address both the airway obstruction and the feeding difficulties of the child. Approximately 70% of patients with airway obstruction can be resolved by adopting a prone position. Similarly, the correct feeding position will resolve most feeding problems. If airway obstruction and feeding problems are not resolved with a change in position, a nasopharyngeal ventilation tube and a nasogastric tube are placed to improve breathing and feeding, respectively. If the airway obstruction is not relieved by non-surgical airway management, surgical treatment is required. The current surgical treatments include: labial-lingual adhesions, mandibular traction osteogenesis, and tracheotomy. Polysomnography and bronchofiberscopy are essential before surgical treatment, the former providing objective indicators of the child’s respiratory status and the latter clarifying the location of the child’s airway obstruction and excluding airway obstruction below the level of the tongue root. The treatment of Piro’s sequence syndrome is a multidisciplinary and comprehensive treatment. The development of the treatment plan revolves around addressing both the airway obstruction and the feeding difficulties of the child. The first step is to assess the type and location of the airway obstruction and the cause of the feeding difficulties. In about 70% of patients, the problem can be solved by adopting a prone position and proper feeding posture. If this treatment is not effective, a nasopharyngeal ventilation tube and a nasogastric tube are placed to improve breathing and feeding, respectively. If non-surgical airway management does not relieve airway obstruction, surgical treatment is required. Currently, the main surgical treatment methods are: lip and tongue adhesions, mandibular traction and osteotomy, and tracheotomy. The polysomnography and bronchofiberscopy are essential before surgical treatment. The former provides objective indicators of the child’s respiratory status, while the latter can clarify the location of the child’s airway obstruction and exclude airway obstruction below the level of the tongue root.