Care of an infant with myelomeningocele may begin with prenatal diagnosis, which allows for optimal perinatal care. In the newborn period, the decision for surgical treatment of myelomeningocele must be made promptly, but only after rational discussion with, and consent by, the affected child's family. Advances in newborn care and in surgical techniques, and an improved ability to both detect and treat hydrocephalus and CSF infections, have resulted in better outcomes for these children. Recent insights into the pathogenesis of myelomeningocele suggest that prevention of most of these congenital malformations may be possible through periconceptual vitamin supplementation.