The vertebral artery originates from the subclavian artery and is divided into four segments (V1–V4). In its intracranial segment (V4), the two vertebral arteries join to form the basilar artery, an unpaired medium-sized artery. However, apart from this typical description, several anatomical variations may occur in the human body. Although in some cases such variations may be asymptomatic, they may be also associated with several pathological conditions, neurological complications, surgical complications, and increased risk of developing vascular diseases. Therefore, it is crucial to obtain sufficient information on the anatomy and variants of both arteries to prevent such complications and ensure the safe completion of surgical and radiological treatments. For this reason, we reviewed studies published up to January 2022 concerning the reported variations of the vertebral artery and basilar artery regarding their origin, course, length, and diameter. We believe that the thorough presentation of these variations would help surgeons worldwide during their daily clinical and surgical practice.