One hundred and two patients with esophageal atresia possessed 237 additional anomalies. Risk grouping of these patients was useful and showed a direct relationship between the increased incidence of anomalies, low birth weight, and nonsurvival. Fifty-seven per cent of the patients with additional anomalies possessed multiple anomalies, most frequently occurring in the gastrointestinal, cardiovascular, and musculosketetal organ systems. Fifty-five per cent of the deaths appeared to result from the coexistent severe anomaly, while the remaining 45% of the deaths appeared to occur as a result of the presence of esophageal atresia. It is our suspicion that the insult producing esophageal atresia with a high incidence of associated anomalies occurs at a more critical time in organogenesis than that event that produces the esophageal atresis alone or with one moderate anomaly.