The authors discuss echocardiographic diagnosis and endovascular treatment of two different anatomical types of pulmonary arteriovenous fistula. Transoesophageal echocardiography in a cyanotic 3 year old boy revealed a direct right pulmonary artery left atrial fistula, treated by surgical ligation of the anomalous feeding artery. A cerebral abscess in a 22 year old man with hereditary haemorrhagic telangiectasia revealed pulmonary and cerebral arteriovenous malformations, which were both treated successfully by embolization. The value of echography is the demonstration of the right to left shunt at pulmonary level and, in some cases, the direct visualization of the arteriovenous malformation. Embolization should be considered as the therapeutic method of choice in all pulmonary arteriovenous fistula, except for direct pulmonary artery left atrial fistula.