Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Chiari II malformation, bowel and bladder dysfunction, skeletal deformations, and neurocognitive impairment. Experimental studies provide compelling evidence that the neurological deficits associated with MMC are not simply caused by incomplete neurulation but rather by the prolonged exposure of the vulnerable neural elements to the intrauterine environment. MMC is the first non-lethal anomaly considered for fetal surgical intervention, necessitating a careful analysis of risks and benefits. Retrospective and prospective randomized studies suggest that fetal surgery of MMC before 26 weeks of gestation may preserve neuromotor function, reverse hindbrain herniation, and reduce the need for ventriculoperitoneal shunting. However, these studies also demonstrate that fetal surgery is associated with significant maternal and fetal risks. Consequently, additional research is necessary to further elucidate the pathophysiology of MMC, to define the ideal timing and technique of fetal closure, and to evaluate the long-term implications of prenatal intervention.