In this work the clinical features, diagnostic methods, surgical procedures and postoperative follow-up are reviewed in 19 cases of atrioventricular septal defect with associated tetralogy of Fallot. The clinical situation was always related to the degree of pulmonary ischemia, that pointed out the urgency and need for a palliative surgical procedure before corrective surgery. There was no data in the ECG for the differential diagnosis of isolated forms of atrioventricular septal defect. In 50% of the cases the cardio-thoracic index was increased by right atrial enlargement. The diagnosis of the malformation was made in every case by means of 2D echocardiography. In 16 cases palliative surgery was done (aortic-pulmonary shunts), with one death (6.2%) due to facts unrelated to the technique. Of the 6 cases with corrective surgery (5 cases with previous shunts), one needed a mitral valve replacement (St Jude prosthesis). There was no mortality in this group and the functional status in the long-term follow-up was: 3 cases were in the functional class I and 3 cases in class II. The overall mortality for all surgical procedures carried out was 4.3%. In conclusion, we consider the use of the corrective surgery adequate to the clinical stability of the patients with atrioventricular septal defect with associated Fallot's tetralogy.