Neuromyelitis optica (NMO) is a demyelinating, autoimmune, and inflammatory disease of the central nervous system characterized by attacks of optic neuritis and transverse myelitis extensive. The presence of a specific antibody called NMO-IgG in serum has been of great help not only for differentiation with multiple sclerosis but to help define the spectrum of NMO. This IgG1 antibody acts on the water channels (aquaporin 4) at the foot of astrocyte. Seropositivity in the NMO spectrum relates to the high probability of relapses. Therefore, preventive therapy with immunosuppressant drugs should be instituted as early as possible because the disability is related to the residualeffect of the attacks. We report a case of a patient with extensive longitudinal myelopathy that does not meet criteria for definite NMO and multiple sclerosis where positive NMO-IgG antibody led to decision to initiate immunosuppressive therapy. We perform a brief review of the topic.