A 44-year-old woman with progressive cervical myelopathy and central cord syndrome was noted to have an extensive cervical intramedullary contrast-enhancing lesion on magnetic resonance imaging (MRI). The lesion resembled a spinal astrocytoma or ependymoma that required surgical intervention. She was subsequently diagnosed to have neuromyelitis optica (NMO), a rare idiopathic inflammatory demyelinating disorder, when the clinical examination revealed left optic atrophy. This was confirmed by a test showing seropositivity for NMO-immunoglobulin (IgG). Disease control was achieved with corticosteroids and immunosuppressive therapy. We report a rare case of a patient with NMO who had MRI features that could have easily led to the condition being misdiagnosed as a spinal cord tumor. The importance of careful history taking, awareness of typical radiological findings and the usefulness of serum NMO-IgG as a diagnostic tool are emphasized.