A full-term infant developed stridor, respiratory distress and hypercarbia shortly after birth requiring urgent airway intervention. The patient underwent urgent intubation via rigid bronchoscopy. The diagnosis of congenital subglottic cyst was made. The cyst was decompressed and the patient was extubated the following day. On repeat laryngoscopy 1 month later, there was no residual disease and the patient remained symptom free. Congenital subglottic cysts are extremely rare and the diagnosis can be either missed or misdiagnosed with more common causes of stridor, such as laryngomalacia. Subglottic cysts may cause total airway obstruction and even death if they are large enough and not treated immediately.