We present herein 10 cases with fetal anomalies of central nervous system (CNS), diagnosed antenatally by ultrasound, at Shimane Medical University Hospital. Five anencephalies, 4 hydrocephaluses and one encephalocele could be diagnosed in utero, but associated 3 sacral meningoceles were failed to detect antenatally. Eight cases were stillbirth and one case with hydrocephalus died due to heart anomaly on 142th neonatal day. But, last case with hydrocephalus was still alive under the pediatric management. We discussed the ultrasonographic findings of fetal anomalies of CNS and reviewed the previous papers.