Rationale:Congenital high airway obstruction syndrome (CHAOS) is defined as complete or partial obstruction of the fetal upper airways. Laryngeal atresia is the most frequent cause.Patient concern:A male neonate born with poor reactions, weakly spontaneous breathing and cyanosis of the limbs was referred to our hospital.Diagnosis:CHAOS with tracheoesophageal fistula.Intervention:A tracheostomy was performed and a 3.0-mm internal diameter tracheostomy tube was inserted.Outcomes:Neonatal survival depended on our immediate postnatal intervention.Lessons:In summary, a multidisciplinary team including otolaryngologists, radiologists, obstetricians, and anesthesiologists must be present during the whole diagnosis and treatment process.