We present the case of a 57-year-old patient who was admitted to the hospital for preoperative cerebral angiography because of an intraspinal mass at the level of C1 and C2. Angiographic examination revealed an abnormal origin of the right vertebral artery, which normally originates from the right subclavian artery. Thus, the right vertebral artery was the last branch of the supraaortic vessels. We also review herein the incidence of the various anomalous origins of the right vertebral artery in the literature and discuss their potential embryologic development and clinical significance.