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Abstract Objective To describe the prenatal diagnosis of Galen vein aneurysm (GVA) based on ultrasonography and magnetic resonance imaging (MRI) in a series of cases, as well as its postnatal outcomes and follow-up until 4 years of age. Methods A retrospective longitudinal study was performed, analyzing a database comprising seven cases of prenatal diagnosis of GVA at two Brazilian institutions from February of 2000 to May of 2012. The following data were evaluated: gestational age at diagnosis, GVA dimensions on ultrasonography, associated fetal changes, findings on fetal echocardiography, gestational age at delivery, type of delivery, birth weight, Apgar score at the 1st and 5th minutes, neonatal outcomes, and survival with follow-up until 4 years of age. Results The mean gestational age ± standard deviation on the prenatal diagnosis of GVA based on ultrasonography was 25±4.9 weeks. The mean length of GVA was 3.2±0.4 cm. The mean gestational age at birth was 37.5±0.7 weeks, and a cesarean section was performed in 85.7% of the cases (6/7). The mean birth weight was 3,070±240.4 g. The total survival rate was 42.8% (4/7), with three neonatal deaths. Of the four survivors, three presented with normal neuropsychomotor development until 4 years of age and only one showed serious neurological sequelae. Ultrasonography and MRI showed similar findings for all seven cases. Conclusions Galen Vein Aneurysm is associated with a high neonatal death rate. Therefore, its prenatal diagnosis is essential for parent counseling and follow-up at tertiary care institutions.