Long-term outcome of 25 fetuses with ventriculomegaly diagnosed before delivery who survived the neonatal period up to school age was examined at 10.1 (standard deviation, 2.6) years of age. Twelve children had normal motor and mental development, seven were severely handicapped, and six showed intermediate outcome. The fetuses with severe handicaps on long-term follow-up had more severe ventricular dilation than the fetuses with good long-term outcomes. Eighteen of the children had surgical treatment, and seven of them had normal long-term development. Thirty percent of surviving fetuses with ventriculomegaly have poor long-term outcomes. In isolated progressive ventriculomegaly, early delivery as soon as fetal lung maturity allows might be reasonable for achievement of better long-term prognosis.