This study aimed to report a case of infant who presented with a prenatal ultrasound diagnosis of agnathia, polyhydramnios followed by a review of previous studies. As widely acknowledge, agnathia is a complex lethal malformation characterized by absent mandible, microstomia, microglossia and ear anomaly which are secondary results to a defect of the first branchial auh. Newborn infants with agnathia often suffer from airway obstruction, causing fatal respiratory failure. The most difficult part of treating those newborns is to keep the airway patent. Therefore, as early airway management planning as possible is the most important part. Airway management was achieved with tracheotomy through an ex utero intrapartum treatment procedure(EXIT). The case of this infant, reporting here, was underwent tracheotomy with preservation of uteroplacental blood flow and gas exchange.