OBJECTIVES: Neuromyelitis Optica (NMO) has been described as a disease clinically characterised by severe optic neuritis and transverse myelitis. There are very few epidemiological studies in NMO and no randomised controlled studies that guide therapy. The aim of this review is to determine epidemiology of NMO and to provide an algorithm of treatment. METHODS: A systematic search was conducted of the relevant published evidence from Embase, MEDLINE, and Cochrane. Search limits were articles in English and in human. Retrieved citations were screened by two independent reviewers according to inclusion criteria: NMO, incidence, prevalence, and treatments reported in population base and observational studies. The analyses of comparable outcomes were carried out as per appropriate statistics along with critical appraisal of the studies. RESULTS: A total 16 studies met the inclusion criteria including six studies reported epidemiological data while 10 other studies reported treatment algorithm. Incidence of NMO ranged from 0.05 per 100,000 per year in United Kingdom (UK) to 0.4 per 100,000 in Southern Denmark. Prevalence was ranged from 0.44 per 100,000 in UK to 4.4 per 100,000 in Southern Denmark. Peak prevalence of NMO occurs among the people at 40-49 years of age. Low level evidence recommended methylprednisolone 1g/day for 3 to 5 days or 2 to 3 sessions of plasmapheresis per week, up to 7 sessions for acute attacks of NMO. Nine studies observed the improvements in the reduction of mean annualized relapse rate. CONCLUSIONS: There is limited evidence on current available treatment therapies for NMO. The available low level evidence found that high dose intravenous corticosteroid pulse and plasmapheresis may help in acute attacks of NMO. Further well designed, adequately powered studies are required in this context.