Case presentation: We report a case of a premature infant with a vein of Galen malformation who presented immediately after birth severe high-output heart failure, pulmonary hypertension and respiratory distress. An echocardiogram showed normal cardiac anatomy and patent foramen ovale. Head ultrasonography and contrast computed tomography of head revealed the aneurysmal dilatation of the vein of Galen and arterial-venous fistulas. Artificial ventilation, inotropic and diuretic i.v. treatment and subsequent orally was initiated. In evolution, the patient presented severe heart failure associated with growth failure and pulmonary infection. Endovascular embolization of the feeding vessels with NBCA was successfully performed at the age of 3 months with an excellent outcome.