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Anatomical variations in the course of the vertebral artery have been previously described in the literature. Generally, these predictable patterns of variations commonly observed in lower cervical vertebral artery anatomy and less commonly described for upper cervical vertebral artery anatomy. Due to presence of these variations, treatment options for upper cervical spine pathology may be influenced and sometimes prevent commonly performed stabilization procedures. Herein author presented a case of vertebral artery anatomic variation at the craniovertebral junction and management option for such variations.