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Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk.