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Neural tube defects are important determinants of perinatal morbidity and mortality, and the risk of death depends on the severity of the lesion and other factors, such as the availability of prior medical and surgical resources. In Brazil, according to the National Information System, in 2016 there were a total of 2,857,800 births, of which 4,085 were born with congenital malformations of the central nervous system, with 735 cases of spina bifida. In the municipality of Rio de Janeiro, of the 89,480 live births, 960 were born with a congenital anomaly of the central nervous system, statistically described, apart from cases of spina bifida, which accounted for 30 in total (BRASIL, 2017). The aim of this book is to review the subject of malformations of the central nervous system with an emphasis on myelomeningocele. It analyses embryonic alterations, clinical and surgical procedures, family relationships, socioeconomic status, quality of life and psychological aspects.