A male full-term neonate is described in whom cardiac insufficiency developed within 24 hours post partum. Ultrasound revealed an arterio-venous fistula of the vein of Galen. The patient's condition did not allow surgical correction and he died on the 22nd day of life. Persistent pulmonary hypertension was an important accompanying feature. The literature is reviewed with respect to the prognosis and the up to now seldom reported complication of persistent pulmonary hypertension of the newborn (PPHN).