Tracheoinnominate artery fistula is a rare disease with an extremely fatal course. Without aggressive surgical treatment, the mortality rate is nearly 100%. We report a 14-yearold male who suffered from acute respiratory distress syndrome as the complication of tracheoinnominate artery fistula. We concluded that ligation of the innominate artery plus iliofemoral arterial bypass and tracheal repair could cure this life-threatening disease, without recurrence. Furthermore, extracorporeal membrane oxygenation can provide adequate pulmonary and cardiac support to help these patients get through the critical postoperative condition.