This article describes an 11-year-old girl with a diagnosis of Ebstein's anomaly. Glenn and Fontan surgeries were performed successfully. She had a generalized tonic-clonic seizure after peripheral intravenous infusion under pressure. A computed tomography brain scan performed 30 minutes later showed multiple serpiginous hypodensities in the cortical sulci and in the superior longitudinal sinus compatible with cerebral venous gas embolism. At follow-up 1 month later, the girl had severe motor impairment. Cerebral gas embolism should be considered in a patient with risk factors and acute neurological symptoms in order to select the treatment of choice, hyperbaric oxygen, and reduce damage to brain tissues.