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Düzce Üniversitesi, Tıp Fakültesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, Düzce, Türkiye Myelomeningocele is the most common significant birth defect involving the spine. It is the most severe form of spinal dysraphism. Myelomeningocele a congenital defect of the central nervous system in which a sac containing part of the spinal cord and its meninges protrude through a gap in the vertebral column; frequently accompanied by Chiari II malformation and hydrocephalus. This segment of the spinal cord and corresponding roots are as a whole non-functional. Genetic and environmental factors are very important for development of this anomaly. Intrauterine repair of the myelomeningocele is an alternative option for preventing of the neurological deficits, Chiari 2, and also hydrocephalus. The aim of this study to present detailed information about myelomenıngocele and its intrauterine repair. Anahtar Kelimeler : Fetal surgery, Tethered spinal cord, Intra-uterin surgery, Hydrocephalus, Myelomeningocele, Neural tube defect, Spina bifida, Spinal dysraphism