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Vascular anatomy is an unreplaceable knowledge for any surgical and interventional specialist, where its proficiency must include at least the most common anatomical variants described in the population. In this subject, aortic arch and its branches are not an exception. The most common anatomical variant of the aortic arch branches is a fourth vessel arising from it, corresponding to the origin of the left vertebral artery. In this case report we describe a patient with an aortic arch branches’ variant plus an intracranial arteriovenous malformation. Up to our knowledge, this is the first case report describing with a proper imaging protocol both a vertebral artery variation and an arteriovenous malformation. Although there is no evidence supporting an association among these two vascular conditions, hereby we present a coincidental vascular finding that requires further research as to propose possible association.