The diagnosis of fetal anomalies is one of the major reasons that legitimat
e an extensive use of ultrasound in pregnancy. Among all fetal anomalies, t
hose pertaining to the central nervous system (CNS) occupy a special positi
on. With an estimated incidence of one per 100 births [1,2] they are among
the most frequent malformations, and they often have a severe prognosis. In
many cases they have a genetic background, and as a consequence of this th
ere is a large number of couples at specific risk who demand antenatal diag
nosis. The CNS was probably the first apparatus to be investigated in utero
by diagnostic ultrasound. Anencephaly was the first congenital anomaly to
be recognized by this technique before viability [3]. Modern high-resolutio
n ultrasound equipment yields a unique potential in evaluating normal and a
bnormal anatomy of the fetal neural axis from the very early stages of deve
lopment. In this article, the most recent advances in the sonographic inves
tigation of the fetal brain and the identification of CNS anomalies will be
reviewed.