A new case of intracranial arteriovenous malformation was observed in a newborn infant presenting as severe congestive heart failure, massive cardiomegaly and intracranial murmur. Cerebral echotomography through the fontanelle showed a large echo-free structure behind the third ventricle. Cerebral arteriography confirmed the vein of Galen aneurysm. A neurosurgical therapy clipping the nutrient vessels had not allowed this child to survive. Diagnostic value of ultrasound examination is emphasized. Surgical therapy is discussed.