Most patients are of normal intelligence and have normal life spans.
A twenty three day old male child with a unilateral left epibulbar dermoid, iris coloboma and a left preauricular pit consistent with Goldenhar syndrome presented with a sudden onset of lethargy and depression of the central nervous system. Diagnostic work up revealed coarctation of the aorta, acute hemorrhage of the left side of the brain. The child died at surgery due to uncontrollable intracranial bleeding. The postulated cause of death was rupture of the malformed meningeal vessels secondary to coarctation related intracranial hypertension. Moya-Moya disease was suspected but could not be confirmed pathologically.