Tracheoesophageal fistulas are due to congenital embryonic abnormalities that form a fistula between the trachea and esophagus. About half of the patients have other congenital anomalies such as cardiovascular, genitourinary and pulmonary insufficiency. Most are sporadic, and only a few have a family history. The clinical presentation depends on the anatomic features and severity of the malformed lesion, with most presenting with choking and vomiting after eating, prolonged recurrent lower respiratory tract infections, and occasionally hemoptysis. The symptoms of choking and reflux are more pronounced with feeding or crying. In milder cases, symptoms often do not appear until adulthood. Tracheoesophageal fistula with esophageal atresia is common, with tracheal hypoplasia and a fistula that connects to the esophagus, with the esophagus either blind-end atresia and enlargement in the form of a sac, or tracheoesophageal fistula without esophageal atresia. Newborns with tracheo-esophageal fistula with esophageal atresia may have choking and regurgitation of food and vomiting of pharyngeal secretions. Death is often rapid due to aspiration pneumonia and respiratory distress.