We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and young children, based on our experience over 7 years. From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated by end-to-end, extended end-to-end, or extended end-to-side ascending aorta and aortic arch anastomosis. The mortality rate was 0.6%. In 138 (82%) patients, the pressure in the lower extremity was higher than in the upper extremity. During 6-24 months of follow-up, hoarseness appeared in 6 patients. Echocardiography revealed stenosis of the end-to-end anastomosis in only 2 patients. We suggest that the best option for surgical treatment of coarctation of the aorta with associated cardiac malformations is a one-stage procedure using a median sternotomy approach. It is better to perform extended end-to-end anastomosis or anastomosis between the distal descending aorta and the left wall of the ascending aorta, or to extend this anastomosis to the transverse arch.