**Abstract**

The prevalence of Congenital central nervous system (CNS) anomalies, including those of the brain and spinal cord, is 3 to 6% in stillbirth and 0.14 to 0.16% in live births. Holoprosencephaly, spina bifida, anencephaly, and encephalocele are major neural tube defects (NTD) encountered in clinical practice. Proper management and diagnosis of these conditions mandate a good understanding of their etiology and classification. Research is being conducted to investigate the etiopathogenesis and treatment of these anomalies. In this chapter, we have reviewed the clinical and pathological aspects of the major NTDs and the latest principles of their management.

**Keywords:** the central nervous system, congenital anomalies, Fetal CNS anomalies, Neural Tube Defects
