To evaluate gestational and neonatal outcomes in pregnancies complicated by fetal hydrocephalus. Retrospective analysis of 287 cases of fetal hydrocephalus followed at the Fetal Medicine Unit of the University of Campinas in the period of 1996 to 2006. Mean maternal age was 25 years, mean gestational age at diagnosis was 27 weeks. There were 50 cases of isolated ventriculomegaly, 95 cases of Chiari II malformation and 142 cases of ventriculomegaly associated with other malformations. Preterm delivery and vaginal delivery were more frequent in the group of ventriculomegaly associated with other malformations. Cardiac, skeletal and renal malformations were the most common associated malformations. Cesarean section was common (95%) in the Chiari II group. Fetal and neonatal death occurred more frequently (29 and 68%, respectively) in the group of ventriculomegaly associated with other malformations. Chromosomal anomalies were present in 15% of 165 investigated cases. Fetal and neonatal prognosis and outcome are associated with the presence of associated anomalies and aneuploidy.2826607611