Malformation of the central nervous system is among the most common of the major congenital anomalies. Exact incidence figures are difficult to determine as they vary with geographical location, the population group under the study and epidemiological technique used. The aim of this study is to evaluate the prevalence and frequency of central nervous system congenital anomalies in Maternal and Child Birth Hospital(MCH) in Najran Kingdom of Saudi Arabia. Prospective descriptive study was performed at all maternal referred to Maternal and Child Birth Hospital – radiology department were studied during the period from December 2016 to December 2017 and fetal with the central nervous system were identified . Maternal age, gestational age, parity and types of central nervous system anomalies were recorded in planning questionnaire for each fetus.