Fistulas between a systemic artery arising from the aorta and the pulmonary vein with absence of a branch of the pulmonary artery and without abnormality of the bronchial tree are very seldom. We report the case of a child which had a continuous vascular murmur with heart failure secondary to a left-left shunt. A left inferior lobectomy was performed. The inferior lobe was the site of a huge arterio-venous fistula between an artery arising, from the aorta and the pulmonary veins.