Congenital isolated 'H-type' tracheoesophageal fistula (TOF) is a rare disorder which is difficult to diagnose. Clinical presentation is characterised by a triad consisting of paroxysmal coughing and cyanosis during feeds; recurrent chest infections and failure to thrive; and abdominal distention secondary to gaseous loading of the bowel. It is often difficult to diagnose 'H-type' TOF because the continuity of the oesophagus is not interrupted. The diagnosis is often missed or delayed, leading to complications such as chronic lung disease and failure to thrive.