Introduction Myelomeningocele (MM) is one of the forms of dysraphism. Hydrocephalus secondary to Arnold-Chiari (AC) malformation is responsible for the morbidity and mortality of the disease. The intrauterine repair of MM may reverse AC and limit hydrocephalus progression. Objective The aim of this work is to report the successful outcome of a case of intrauterine correction of MM, with favorable evolution and birth at 36 weeks of pregnancy. Case report KCMC, 19 years old, was submitted to intrauterine repair of MM at 24 weeks of gestational age after approval by the family and the Research Ethics Committee of the School of Medical Sciences-UNICAMP. Conclusion Although it is not the standard option for surgical correction, selected MM cases with early fetal diagnosis may be eligible for intrauterine repair, when there is consensus with the Research Ethics Committees and family members.