A neonate presented with anatomically discrete cerebral arteriovenous fistulae located in the right sylvian fissure and the cerebellar vermis that were initially detected by prenatal ultrasonography. Following delivery of the baby by caesarean section, both malformations were treated by surgical obliteration. These intracranial vascular lesions were associated with cardiac anomalies and a periductal coarctation of the aorta, which was treated with a left subclavian rotational arterial pedicle repair. Follow-up examination of the infant at age 13 months demonstrated an excellent clinical result with normalization of the circulation. The pathophysiology of this syndrome is discussed and the literature reviewed.