A 6-month old girl who had pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals underwent one-stage complete repair with unifocalization through a bilateral thoracosternotomy(clamshell incision). There were no serious postoperative compli cations, and the postoperative echocardio-graphy showed no residual ventricular septal defect or significant pulmonary artery stenosis. In this condition, great surgical variability exists regarding the sources of pulmonary blood flow. Recent clinical work has focused on a one-stage complete repair. The potential advantages of the clamshell incision are apparent in terms of mediastinal approach, postoperative results, and safety.