This chapter provides detail description of Vein of Galen malformations. Chapter has 3 well illustrated diagrams and pictures. Vein of Galen malformations, anatomical considerations and classification, clinical features of patients with vein of Galen malformation, diagnostic investigations of patients with vein of Galen malformation, treatment of patients with vein of Galen malformation and endovascular treatment of patients with vein of Galen malformation are described in detail. 23 references are included at the end of the chapter to facilitate further reading.