Background: Central nervous system (CNS) anomalies are the most serious congenital abnormalities. Ultrasound examination is an effective and non-invasive modality for prenatal diagnosis of these anomalies. The objectives of this study were to detect the proportion of C.N.S malformations in patients attending the feto-maternal unit at Minia University and analyze the related factors at 22nd to 28th weeks of gestation and detect the diagnostic accuracy of Ultrasound in the diagnosis of CNS malformations. Methods: This is a prospective study that was conducted on 100 women who were referred to Fetomaternal Unit at Minia University for any C.N.S anomalies, in the period between September 2019 and September 2020. Results: 35% of cases had a family history of congenital fetal malformations and 8% of cases had a family history of C.N.S fetal malformations. 42% of cases had a history of aneuploidy and 46% of cases had a history of congenital infections. Conclusion: Dimensional ultrasonography is useful in detecting and diagnosing some fetal central nervous system abnormalities. However, owing to the location of the fetus, some defects may be difficult to identify with two-dimensional ultrasonography. For example, while acquiring the median plane with 2D transabdominal ultrasonography is difficult and requires specific competence in transvaginal transfontanelle scanning, it is often regarded as a good preliminary diagnosis tool for screening for fetal defects.