Myeloschisis is the most serious and complex congenital anomaly in spina bifida manifesta (cystica). However, with improvements in medical care and increased understanding of its pathophysiology, the associated long-term morbidity and mortality rates have been significantly reduced. This article reviews various issues associated with the neurosurgical management of patients with myeloschisis, such as perinatal management, repair surgery for myeloschisis, neurosurgical management of hydrocephalus, Chiari malformation type II, tethered cord syndrome and epilepsy, and intrauterine fetal surgery.