Congenital vascular malformation (CVM) between the external carotid artery and the internal jugular vein is a rare disease, it originates as a consequence of arrested development during various stages of embryogenesis. The natural history of CMVs is progressive growth, it can remain clinically silent until it progresses causing local swelling accompanied by symptoms and signs of arteriovenous shunting, mass effect, ischemic insult with ulceration of skin or bleeding. In literature only few cases of CMVs have been reported, therefore there is still no consensus about the correct surgical approach to this pathology. We report an uncomplicated case of CVMs in a 15-year-old female successfully treated with coils embolization. To our knowledge, this is the eighth reported case in the literature review.