One hundred ninety-one infants under 1 year of age underwent repair of coarctation of the aortz during a recent 14 year period. All operations were performed on an urgent, nonelective basis. One third of the infants had isolated coarctation (Group I), one third had associated ventricular septal defect (VSD) (Group II), and one third had other complex anomalies (Group III). The operative mortality rate has decreased to 4% in infants with isolated coarctation of the aorta but remains at 25% for repair of coarctation in association with significant intracardiac disease (Groups II and III). The late mortality rate also was affected by intracardiac defects. No late deaths occurred in Group I patients, whereas the 5 year mortality rate was 25% in Groups II and III. Surgical repair was by resection and end-to-end anastomosis in most (92%) cases. Recurrent coarctation, as defined by arm/leg blood pressure gradient at rest, developed in 54% of the survivors within 7 years following operation. Recoarctation did not affect late mortality rate. Twelve children (11.1% of survivors) underwent repair of recoarctation during this interval. Hypertension developed in 27% of the children followed more than 5 years after repair and was always associated with residual arm/leg blood pressure gradient. There are no cases of idiopathic hypertension in this series.