A preterm female presented with severe respiratory distress in the delivery room and was found to have tracheal agenesis with a tracheoesophageal fistula and a congenital heart defect. Tracheal agenesis is uncommon and is often associated with other congenital abnormalities. Although there are surgical options for repair, mortality remains high.
Keywords: Tracheal agenesis; tracheal reconstruction; tracheoesophageal atresia; tracheoesophageal fistula.