OBJECTIVE Detailed knowledge about anatomic variations of the aortic arch and its multiple branches is extremely important to endovascular and diagnostic radiologists. It is often hypothesized that anomalous origin and distribution of large aortic vessels may alter the cerebral hemodynamics and potentially lead to a vascular pathology. METHODS In this article, we describe a case of anomalous origin of the right vertebral artery, which was detected during an intervention. We further reviewed the available literature of anomalous origin of the right vertebral artery. The probable embryologic development and clinical significance are discussed. RESULTS The incidence of anomalous origin of a vertebral artery seems to be underestimated in recent literature. A careful review of the literature shows more than 100 such cases. The right vertebral artery can arise from the aortic arch or one of its branches. Dual origin of the vertebral artery is not uncommon. The embryologic developmental hypotheses are contradictory and complex. CONCLUSIONS Anomalous origin of the right vertebral artery may not be the sole reason behind a disease process. However, it can certainly lead to a misdiagnosis during diagnostic vascular studies. Detailed information is essential for any surgery or endovascular intervention in this location.