The left isomerism heart is common to have various forms of the systemic and pulmonary venous connections, for which numerous reparative methods have been proposed. This report describes a successful extracardiac rerouting of the persistent left superior vena cava of a 6-year-old girl patient having the isomerism heart, bilateral superior vena cava, partial anomalous pulmonary venous connection, and partial form of the atrioventricular septal defect. Intraatrial rerouting of the left superior vena cava was found unfeasible, since this procedure had a potential hazard to obstruct the systemic and/or pulmonary venous drainage. The patient recovered uneventfully and is leading a normal life 6 years after the surgery.