A new technique to correct persistent left superior vena cava (LSVC) drainage into the left atrium is described in a 14-year-old patient with situs inversus, left atrial isomerism common atrium, and mitral valve regurgitation. During surgery, occlusion of the LSVC markedly increased the venous pressure, precluding its ligation. Because of the malposition of the heart and the unusual atrial anatomy, correction with an intra-atrial baffle was not attempted. After correcting the intra-cardiac anomaly, the LSVC was divided and anastomosed to the "right" atrial appendage using a segment of PTFE graft. The postoperative course was uneventful and an angiogram demonstrated excellent performance of the graft.