We report a case of a 5-year-old girl who suffered a massive arterial air embolism during surgical closure of an atrial septal defect. The risk of permanent neurologic deficits or even fatal outcome is significant (mortality rate, 31%). We successfully treated a proven arterial air embolism with intraoperative (retrograde cerebral perfusion) combined with postoperative procedures (deep barbiturate anesthesia and hyperbaric oxygenation). At discharge the girl had fully recovered from the initial neurologic defects.