Vein of Galen aneurysmal malformation (VGAM) is a very rare congenital vascular malformation. Also known as the “median prosencephalic arteriovenous fistula”, VGAM is a subtype of dural arteriovenous fistula. It is believed that the development of VGAM occurs between weeks 6 and 11 of embryo development. Prenatal diagnosis is based on fetal ultrasound, between the second and third trimesters. Despite prenatal diagnosis, this congenital malformation is associated with high morbidity and mortality. We report the case of a 38-year-old primigravida referred at 32 weeks of gestational age to our Prenatal Diagnosis Unit for a suspected VGAM. The remaining fetal assessment was normal. At 38 weeks, a male newborn weighing 3,825 g was born. The postnatal evaluation confirmed VGAM. Endovascular treatment was performed at 4 and 5 months, without complications. The patient is currently 9 months old and has a normal neurodevelopment.