Congenital esophageal atresia with proximal and distal tracheoesophageal fistula occurs very rarely. This report describes late diagnosis of a congenital proximal fistula in a 14-year-old girl who underwent surgical repair of a congenital esophageal atresia with distal tracheoesophageal fistula on her first day of life and suffered recurrent bouts of lower airway infections and chronic cough, with the diagnosis of asthma in later childhood.