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Women with spina bifida in pregnancy require complex multi-disciplinary management. Most women have uncomplicated pregnancies; however, complications are more frequent than in ‘low risk’ pregnancies. Careful antenatal planning and management of the complications can optimise outcome. There are currently no guidelines on the management of pregnant women with spina bifida, but there is a growing body of evidence from case reports and series examining the antenatal and postnatal course of these women. In this review, we examine the available literature and provide a framework on the prenatal counselling, antenatal, intrapartum and postnatal management of pregnant women with spina bifida.