H-type tracheoesophageal fistula (TEF) is extremely rare in infants and children, and clinical manifestations of this condition are diverse based on its severity. Some cases of congenital TEF diagnosed in adulthood have been reported, which indicate the difficulty of early diagnosis of this disease. Gastroesophageal reflux (GER) may induce chronic aspiration, pulmonary aspiration, apparent life-threatening events, and failure to thrive. We report a 5-month- old boy whose recurrent pneumonia and wheezing did not improve under usual treatment and led to acute respiratory distress syndrome. He was found to have severe GER on the second-trial of the esophagogram and was eventually revealed to have congenital H-type TEF upon repeated evaluation.