Craniocervical spine meningiomas are rare. They often present with non-specific motor or sensory symptoms. Presenting symptoms can include gait ataxia, radiculopathy, myelopathy, back pain and sensory deficits. Spinal meningiomas are slow-growing tumours, with an insidious onset. Due to the critical location of craniocervical meningiomas, severe symptoms such as respiratory distress and quadriparesis are possible. We describe the clinical presentation of a craniocervical junction meningioma, its relevant neuroimaging findings, diagnostic challenges and management. A woman in her 30s presented with a subacute onset of neck pain, headaches, paresthesia and a Hoffman’s sign of the left upper extremity. A cervical spine MRI revealed an intradural extramedullary craniocervical junction meningioma involving the C1 segment with cord compression. The tumour measured 1.4×2×2.2 cm. A mid-line suboccipital craniectomy, tumour resection (Simpson grade II) with cervical laminectomy, and dural grafting were completed for definitive management. A brief literature review was conducted yielding a total of 24 cases.