A major development over the past two decades was the recognition of recurrent neuromyelitis optica (NMO) as a particular central nervous system disorder different from multiple sclerosis (MS). Here we reviewed the epidemiology of NMO in Latin America (LATAM). A predominance of a mixed population is found in this region. Recurrent NMO in black women was described in the Caribbean Islands and in Rio de Janeiro. The prevalence of NMO in LATAM varied from 0.37/100,000 (Volta Redonda city) to 4.2/100,000 inhabitants (Caribbean Islands). NMO differs significantly from MS with respect to gender, ethnicity, morbidity and genetic susceptibility. An association of the HLA DRB1*03 alleles with NMO was described in the French Antilles, Ribeirão Preto, Rio de Janeiro and Mexico. It is not common to find familial forms of NMO. NMO represents 11.8% of all inflammatory idiopathic diseases in South America (SA). In SA, the highest frequency of NMO occurs in African Brazilian young women. The overall relative frequency of NMO among MS cases in this region was 14%, decreasing following a north-south gradient, which parallels the percentage of nonwhite people.