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Spina bifida is a defect in which the vertebral arch of the spinal column is either incompletely formed or absent. Failure of closure of the neural tube during the third week of gestation leads to the constellation of defects observed in patients with meningomyelocele or open spina bifida. Myelomeningocele is the most common neural tube defect and the most severe birth defect compatible with long-term survival. It is associated with several characteristic central nervous system anomalies. Leak of cerebrospinal fluid (CSF) is commonly observed. The major indication for early operative repair (within 48 hours of delivery) is prevention of infection. Protection of the exposed neural tissue from trauma and drying is essential. An understanding of the pathophysiology and associated conditions of myelomeningocele helps guide anesthetic management of these patients.