Neruomyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. It is crucial that treatment is started as early as possible to avoid new relapses and further disability. Treatment of NMO spectrum disorders is divided into two objectives: one is to control the inflammatory damage in acute attacks and the other one is a maintenance treatment to avoid relapses. The former is based on high-dose intravenous corticosteroids and plasmapheresis, the latter is based on low-dose corticosteroids and immunosuppressants. High-dose intravenous methylprednisolone is the first-line therapy for acute exacerbations of NMO spectrum disorders. Plasmapheresis should be started soon if corticosteroid is not efficacious. Maintenance therapy is based on low-dose oral corticosteroids and non-specific immunosuppressant drugs, like azathioprine, tacrolimus, mycophenolate mofetil and mitoxantrone. New therapy strategies using monoclonal antibodies like rituximab; an anti-CD20 monoclonal antibody, and eculizumab; an anti-C5 monoclonal antibody, can also prevent relapse in NMO. On the other hand, interferon-beta, natalizumab and fingolimod, a first-line disease modifying drug of multiple sclerosis, is not effective in NMO spectrum disorders. Treatment of the symptom for spasticity, pain, dysuria may significantly improve the quality of life of the NMO patient.