Abstract: Objective To summarize onestage repair of coarctation of the aorta(CoA) with intracardiac malformation in infants. Methods From January 2001 to January 2006, 28 patients with CoA and associated intracardiac malformation underwent onestage repair. There were 18 patients with preductal and another 10 patients with juxtaductal CoA. Six patients were accompanied by diffuse hypoplasia of aortic arch and 22 patients were associated with patent ductus arteriosus (PDA). Associated cardiac malformation included ventricular septal defect in 16 patients, ventricular septal defect and atrial septal defect in 5 patients, atrioventricular canal defect in 3 patients, transposition of great artery and ventricular septal defect in 2 patients and double outlet right ventricle in 2 patients. Left posterolateral thoracotomy combined with median sternotomy were used in 12 patients and single median sternotomy was used in 16 patients. End to end anastomosis of aorta with removal of the coarctated segment were performed in 20 patients, Gore-Tex patch graft aortoplasty were performed in 4 patients and aortoplasty with left subclavain flap were performed in 4 patients. Results There was no death.The lasting time of intubation was 7h to 13d, and intensive care unit (ICU) staying time was 3 to 18d. Four patients received delayed sternal closure in 48 to 72h after surgery because of cardiac dilatation. Three patients received peritoneal dialysis for acute renal failure following cardiac surgery. Three patients received inhalation of nitric oxide for treatment of postoperative pulmonary hypertension. The systolic pressure difference between lower limb and upper limb measured after operation was 10 to 20mmHg in 18 patients.There were no obvious systolic pressure difference between arm and leg in 8 patients.The systolic pressure of upper limb was higher than that of lower limb in 2 patients, and the pressure difference was 15mmHg and 20mmHg. All patients were followed up from 3 to 50 months. They were all survival except one died of severe tricuspid regurgitation, pulmonary infection and respiratory failure 3 months after surgery which diagnosis was transposition of great artery and ventricular septal defect. The results of other 27 patients were satisfactory. Recoarctation occurred in 1 patient with end to end anastomosis and 1 patient with Gore-Texpatch graft aortoplasty. The pressure difference through the measurement of echocardiography was 25mmHg and 28mmHg. No reoperation were undergone. Conclusion CoA with intracardiac malformation can be repaired by single median sternotomy or left posterolateral thoracotomy combined with median sternotomy in onestage. This operative approach is beneficial, not only with shorten period of therapy and less operative cost, but also advantageous for the recovery of cardiac and pulmonary function.