Neuromyelitis optica (NMO) is a rare autoimmune disorder distinguished by optic neuritis, transverse myelitis, and area postrema syndrome. Headaches as an initial manifestation are uncommon and often overlooked. This case describes a 46-year-old female with a 20-year history of migraines. The patient developed cervicogenic headaches three years before presenting with optic neuritis. MRI findings of optic and spinal lesions, together with positive aquaporin-4 immunoglobulin G antibodies, corroborated the diagnosis of NMO. Treatment with intravenous methylprednisolone, azathioprine, and rituximab resulted in substantial improvement in visual acuity and headache intensity. This case emphasizes the significance of investigating secondary causes of cervicogenic headaches and raises awareness about NMO in such patients, highlighting the benefit of early treatment in preventing long-term disability.