BACKGROUND: Cervical myelopathy is rarely caused by vertebral artery (VA) compression, and a very limited number of cases have been published. In most of these cases, dorsal cord compression was observed and treated by microvascular decompression (MVD). However, in the very rare case of ventral spinal cord compression by the VA (VSCV), access for MVD is significantly limited. After encountering such a case, the authors systematically reviewed and analyzed the available published clinical experience and describe the selected surgical strategy for VSCV. OBSERVATIONS: A 78-year-old woman presented with progressive numbness of the right upper extremity, neck pain, and gait disturbance. On MRI, the spinal cord was significantly compressed ventrolaterally at the C1 level by the left VA without other anomalies. A systematic review identified only one similar case of VSCV treated by dural grafting and epidural fat packing. An occipital craniectomy and C1 laminectomy were performed. After dural incision, the uppermost two pairs of dentate ligaments were divided, the spinal cord was mobilized, and extended duraplasty was performed. Symptoms gradually improved and MRI confirmed sufficient decompression. LESSONS: VSCV can be effectively treated by decompression, denticulotomy, mobilization of the spinal cord, and craniovertebral canal expansion with duraplasty, without direct MVD. https://thejns.org/doi/10.3171/CASE24727.