Patients who have undergone repair of esophageal atresia and tracehoesophageal fistula as infants have been noted to have residual esophageal dysmotility and pulmonary dysfunction during their childhood years. However, limited information is available about the long-term follow-up of these patients. In this study we performed esophageal and pulmonary function studies on 12 adults who had required surgical repair of these defects in the first week of life. Most patients had symptoms of dysphagia and heartburn at time of evaluation. Pathologic gastroesophageal reflux was documented in 67% of patients and esophagitis was noted in 34%. All patients had esophageal motility abnormalities characterized by low-amplitude nonperistaltic waves throughout most of the esophagus. In addition, although most patients had no respiratory symptoms, mild restrictive lung volumes were noted in many patients. However, airflow obstruction and airway hyperreactivity were not present. These data demonstrate that clinical symptoms and abnormal esophageal manometry and pulmonary function persist well into the third and beginning of the fourth decade after repair of esophageal atresia and tracheoesophageal fistula in infancy.