The acronym Vertebral abnormalities, Anal atresia, Tracheo-esophageal fistula with Esophageal atresia, Radial and Renal dysplasia was described in 1973 and later updated to VACTERL with the inclusion of Cardiac anomalies and Limb defects rather than radial anomalies. The most commonly associated cardiac anomalies are ventricular septal defects, and tetralogy of Fallot (TOF), but a variety of simple and complex cardiac defects are possible. Patients with VACTERL association frequently require surgery for anal atresia or repair of esophageal atresia/tracheoesophageal fistula in the first days of life. Patients with congenital heart disease have significantly more complications, including difficulties with ventilation and oxygenation, need for inotropic therapy, longer duration of mechanical ventilation, and longer intensive care and hospital stays. Patients with ductal-dependent cardiac lesions are an even higher risk group. The perioperative management of a premature infant with unrepaired TOF for repair of esophageal atresia and tracheoesophageal fistula is discussed in this chapter.