One hundred consecutive patients with esophageal atresia or tracheoesophageal fistula, or both, were treated at The Children's Mercy Hospital during the past 14 years. Each patient was evaluated and a plan for therapy was formulated depending on the type and urgency of concomitant disease. Healthy patients were treated by primary repair. Those with respiratory disease were treated before repair by gastrostomy, upper pouch suction, and antibiotics. In the small premature infant and the patient with major associated anomalies, repair of the esophageal atresia and tracheoesophageal fistula was postponed by gastrostomy, upper pouch suction, and parenteral nutrition while the concomitant disease was treated. Fifteen patients with life-threatening associated anomalies required operation before esophageal repair and 13 of them survived. During the initial hospitalization two deaths related to esophageal atresia occurred, whereas three prerepair and two late (2 1/2 and 3 months) postrepair deaths related to associated anomalies occurred. Thus, 93 of the 100 patients left the hospital alive. The increased survival is due not only to improved care of esophageal atresia and tracheoesophageal fistula, but also to a more aggressive approach to the treatment of the serious associated anomalies.