Fenestration of the vertebral artery is a rare vascular anomaly and most commonly observed in the extracranial segments of the vertebral artery. The theory of its etiology involves different stages of embryological development of the vertebral artery. According to the documents, vertebral artery fenestration may be related to other vascular anomalies. Anatomical variations of the vertebral arteries can pose significant risk for vascular injury during surgical and endovascular interventions. Therefore, it is important to understand and recognize these anomalies, and care should be taken to avoid unintentional injuries.