The vein of Galen malformation is a rare midline intracranial arteriovenous malformation. The majority of the malformations were seen in neonate, infancy and childhood, and the clinical symptoms and prognosis depended on age of presentation. The authors report a case of the vein of Galen malformation in 17 month-old female with hydrocephalus, which is confirmed by computed tomography and digitla subtraction angiogrpahy.