Modern ultrasound equipments allow a detailed investigation of the fetal brain from a very early stage of development. Congenital anomalies arising from or involving the central nervous system can be accurately predicted. On the basis of their personal experience, which includes 48 cases, and of a review of the current literature, the authors discuss criteria for the intrauterine diagnosis and obstetrical management of intracranial anomalies such as hydrocephalus, holoprosencephaly, cephaloceles, porencephaly, hydranencephaly, and microcephaly.