Variations in vertebral artery origin and course are well-described in the literature. The origin of right vertebral artery from the right common carotid artery is an extremely rare variant. We describe a unique case of a child with Down syndrome with variant origins of bilateral vertebral artery, an aberrant right subclavian artery and concomitant Moyamoya disease of intracranial circulation. The presence of variations of the origin and course of craniocervical arteries might have profound implications in angiographic and surgical procedures and hence it is of great importance to be aware of such a possibility.