We present a case of posterior atlantoaxial screw-rod fixation in a patient with an aberrant vertebral artery (VA) course combined with bilateral high-riding VA. An aberrant VA which courses below the posterior arch of the atlas (C1) that does not pass through the C1 transverse foramen and without an osseous anomaly is rare. However, it is important to consider an abnormal course of the VA both preoperatively and intraoperatively in order to avoid critical vascular injuries in procedures which require exposure or control of the VA, such as the far-lateral approach and spinal operations.