Dural arteriovenous malformation (AVM) is a rare clinical entity, presenting most commonly in adult patient with pulsatile tinnitus or haemorrhage. We report a case of a neonate with a massive dural arteriovenous malformation (AVM) with signs of cardiac failure soon after birth. The diagnosis was first suggested during angiocardiography when a hypertrophied left common carotid artery was detected. The AVM was supplied by branches from the left external carotid artery and the left veretebral artery and drained into the left transverse sinus near the torcula. This was excised in two stages. Post-operative angiography showed no residual malformation. The patient was well and developing normally 4 years after surgery. Review of the literature showed only 2 similar cases of dural AVM presenting in the neonatal period, both patients died, reflecting the high risks involved and the difficulties encountered in the management. Ligation of arterial supplies can be a useful temporary procedure in these high risk babies. Definitive excision should be performed later when the baby grows stronger.