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Neural tube defects, with a birth incidence of approximately 1/1,000, are the second most common (after congenital heart defects) type of congenital birth defects. The most common clinical presentations of neural tube defects include spina bifida and anencephaly. Spina bifida, which is one of the most common, serious malformations of human structure, is a general term that encompasses a wide range of malformations. With constant improvements in medical and surgical care, and subsequent decreases in spina bifida-related mortality and morbidity many women with these defects are reaching childbearing age. Provision of labor analgesia in pregnant women with spina bifida continues to be a challenge. This manuscript attempts to provide a synopsis of the available literature on labor analgesia in pregnant women with spina bifida.