Complex unroofed coronary sinus with a persistent left superior vena cava has as its commonest major associated intracardiac anomaly a partial or complete atrioventricular canal defect. In this clinical setting, biventricular repair with construction of a complex intra-atrial baffle from the pulmonary veins to the mitral valve has a reported mortality rate of as high as 50%. Looking for an improvement, we have carried out an extracardiac repair of the anomalous systemic venous component with atrial septation. In 2 infants (aged 7 and 12 weeks) with unroofed coronary sinus, bilateral superior venae cavae, right isomerism, and complete atrioventricular canal, in addition to patch closure of the ventricular component of the atrioventricular septal defect, a baffle was constructed between the pulmonary veins and the mitral valve. In four subsequent infants (aged 7,10,16, and 20 weeks) with unroofed coronary sinus, bilateral superior venae cavae, complete atrioventricular canal, right isomerism (n = 2), and mild infundibular stenosis (n = 1), repair consisted of end-to-side anastomosis of the left superior vena cava to the right superior vena cava and complete repair of the atrioventricular canal and associated conditions. There was no mortality. The early postoperative course in the two patients with intra-atrial baffle was characterized by increased left-atrial pressure (18 and 20 mm Hg), with varying degrees of pulmonary venous congestion, supraventricular tachycardias, and systemic hypotension. The pulmonary venous congestion increased, so that one patient was successfully converted 10 weeks postoperatively to an extracardiac repair with septation of the atria and the other will probably follow. In the 4 patients with a primary extracardiac repair, the hemodynamic result was excellent, with a median left-atrial pressure of 11 mm Hg on the first postoperative day. At a median follow-up of 12 months, all 5 patients with an extracardiac repair are clinically well with widely patent anastomoses between the left and right superior venae cavae. The extracardiac repair technique for complex unroofed coronary sinus, as opposed to the intra-atrial baffle repair, avoids creation of a small and low-compliance left-atrial compartment with the potential for development of pulmonary venous congestion.