Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.
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