Neural tube defects are the most common congenital malformation of the central nervous system. The primary focus of this article will be to discuss their epidemiology, etiology, manifestations, complications, management, and prevention. The primary abnormalities of myelomeningocele are hydrocephalus, Chiari II malformation, and spinal cord dysplasia. This article will discuss the management of the major clinical manifestations, such as bowel and bladder dysfunction, various orthopaedic complications including lower extremity deformities and scolisosis, and cognitive abnormalities. Rehabilitation of youth with myelomeningocele must be developmentally based and comprehensive, including psychosocial issues. Perinatal use of folic acid has been effective in reducing the incidence of neural tube defects. Last, there will be brief discussions on prenatal diagnosis and intrauterine surgical repair of myelomeningocele.