INTRODUCTION: The caudal end of the human embryo is a major site of multipotential cells. We now present two cases of simultaneous arteriovenous malformation and lipomyelomeningocele and review the literature regarding this potential association. DISCUSSION: In the context of rarity and following a review of the literature, both an arteriovenous malformation and lipomyelomeningocele in the same patient appears to be more than serendipitous. The natural history of these vascular malformations in patients with concomitant lipoma or lipomyelomeningocele cannot be determined from such a small group. However, only one patient in our review was found to have consequences thought to be related to their arteriovenous malformation, which was in the upper thoracic region and not related to a lipomyelomeningocele.