Congenital long-segment tracheal stenosis with complex cardiac anomaly has generally been regarded as a fatal disease This report described the successful concomitant repair of unexpected congenital tracheal stenosis and complex cardiac anomaly with the use of edrdiopulmonary bypass. The patient was a 3-month-old girl with coarctation of aorta, V D, and PDA. The presence of tracheal stenosis was not discovered until when difficulty with endotracheal intubation was encountered at operating room. Thus, we decided concomitant repair of both lesions and performed anterior pericardial tracheoplasty combined with one stage repair of coarctation of aorta, VSD, and PDA under the cardiopulmonary bypass. The patient is doing well without any signs of complication at present, 2 years and 1 month after the operation.