The authors report a case of a 38-year-old man with an esophageal squamous cell carcinoma after repair of esophageal atresia with tracheoesophageal fistula. This carcinoma occurred at a young age, near to the scar of the old anastomosis, in a patient with no other apparent risk factors. It is hypothesized that stasis caused by impaired esophageal motility may be the underlying cause. A single case is not enough to unequivocally prove a possible relationship between esophageal atresia and the development of esophageal cancer. Now that the first generation of survivors of esophageal atresia is reaching middle aged adulthood, one should, however, be aware of a possible increased incidence in these patients. Copyright (C) 2001 by W.B. Saunders Company.