Intracranial arteriovenous malformation is rarely presented in newborns or infants. We describe an unusual case of congenital arteriovenous malformation of the brain with multiple sequestered grape-like venous sacs presented with congenital hydrocephalus. This 4-month-old girl born with a large head, presented with progressive hydrocephalus over a period of 4 months. The brain CT showed multiloculated cysts with a high-density mural nodule and thin cerebral mantle. The right lateral ventricle was collapsed by the cystic lesion, and the contralateral ventricle was markedly dilated, which was thought to be due to aqueductal obstruction by the conglomerated nidus of the arteriovenous malformation. Surgical removal of both hemispheric masses, including the overlying thin mantle. The lesion was pathologically confirmed as the arteriovenous malformation which was composed of markedly dilated veins and multiple sequestered aneurysmal sacs. The overlying cerebral tissue was dysplastic and partly infarcted. This case shows that intracerebral arteriovenous malformation is indeed a congenital anomaly and suggests that intrauterine vascular compromise can result in focal or wide maldevelopment of the brain.