Vein of Galen malformation (VGM) is a high-flow intracranial vascular malformation, presenting in utero or after birth with a range of initial symptoms. The natural history of untreated VGM is grave, with progressive permanent neurological dysfunction, developmental delay, seizures, intracranial hemorrhage, and death. Presentation varies with age, with more severe manifestations resulting in earlier presentation and poorer outcome. The mainstay of treatment is medical stabilization followed by endovascular embolization. The goal is to reduce flow through the malformation such that normal development can proceed without neurological deficit. The past 20 years have seen great advances both in the technical treatment of the vascular lesion and in the overall prognosis of patients treated for this condition. Key to a good outcome is appropriate selection of patients and treatment by physicians familiar with this pathophysiology.