During the period Jan 1978 to Dec 1988, 41,867 deliveries took place at the University of Chile Hospital. Among them, 148 babies were found to have malformations of the central nervous system, an incidence of 3.6 per 1000 live births. A longitudinal study from 1969 to 1988 suggests a yearly increment of 0.1% in the incidence rate of these malformations. The comparison of some quantitative variables, such as gestational age, birthweight, number of previous abortions and some risk factors like maternal illness, bleeding, radiation exposure, drug ingestion during the first trimester of pregnancy and instructional level of both parents show significant differences between the malformed and the control newborns. No significant differences were found for maternal age, sex nor seasonal variation.