The exact cause of TEF remains unknown, but it is believed to result from genetic and environmental factors. TEF often occurs alongside esophageal atresia, where the upper esophagus does not connect with the lower esophagus and stomach. These conditions are part of the VACTERL association, which includes vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities.