Abstract Vertebral artery fenestration (VAF) is a type of anomaly of the vertebral artery in which a segment of the vessel divides into two separate channels that join together later in the course. Neurenteric cysts are rare, congenital lesions arising within the spinal canal usually in the cervical region. Vertebral artery anomalies if not considered during operative planning can lead to iatrogenic injury. We report a case of a 5-year-old boy who presented with a history of acute onset, non-traumatic spastic quadriparesis. Examination revealed the presence of cervical neurenteric cyst present ventrally at C1–C3 level. Further workup revealed the presence of bilateral VAF with the fenestrated part going along the inferior aspect of the arch of atlas bilaterally. Surgical excision of the lesion was done taking into consideration the anomalous course of vertebral artery. VAFs are classified according to their location as intracranial or extracranial and according to the segment of the vertebral artery from which they arise (V1–V4). Iatrogenic injury to the vertebral artery has been reported during both anterior and posterior approaches to the cervical spine. Due consideration should be given to anomalous course of vertebral artery while planning the surgical approach in craniovertebral junction.
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