Spina bifida remains a challenging neurosurgical entity to manage despite both an increased awareness of the disease as well as a decreased incidence due to folic acid supplementation. We review the spectrum of neural tube defects, which are the second most common serious congenital defect and the most common of the central nervous system, and discuss the latest management paradigms. The challenges of timely diagnosis and treatment of spina bifida occulta and the latest advances in fetal repair of spina bifida aperta (myelomeningocele) will be discussed. The authors review the literature and share their experience with managing neural tube defects.