During a 10-year period, 313 infants were treated at the Hospital for Sick Children for esophageal atresia, of whom 46 had features of the VACTERL association. These 46 patients had a mortality rate of 24%, most deaths being caused by cardiovascular abnormalities. Infants weighing less than 2,050 g had a mortality rate of 26%, double that of heavier infants. During the first year of life there was considerable morbidity resulting from the tracheoesophageal abnormalities. Whenever possible primary repair of the esophagus should be performed; however, many patients with VACTERL association had an unusually long gap between the two ends of the esophagus, and repair under tension was associated with a high incidence of complications. Of the 35 survivors, 25 are in a satisfactory, stable condition, but 10 still require treatment necessitating repeated admission to hospital. Anorectal, renal or skeletal anomalies rarely caused death, but, when severe, were associated with a poor quality of life.