Abstract Objective Esophageal atresia (EA) predisposes to gastroesophageal reflux that is associated with intestinal metaplasia and may result in development of adenocarcinoma of the esophagus. To date, the literature has reported 5 cases of esophageal cancer in adult patients treated for EA. The aim of this study was to find out the incidence of esophageal cancer in adult patients with repaired EA. Patients and Methods Five hundred two consecutive patients treated for EA from 1949 to 1978 were followed-up for cancer through the files of the population-based countrywide cancer registry from 1967 to 2004. The number of cancer cases observed and person-years at risk were counted, and the expected number of cancer cases estimated from the national cancer incidence rates. The standardized incidence ratios (SIRs) were calculated by dividing the number of cancer cases observed by the expected numbers. Results None of the 502 patients were lost to follow-up; 230 patients who died before 1967 younger than the median age of 8 days were excluded from further analysis. The 272 remaining patients (142 males) were eligible for follow-up (median age, 35 years; range, 2 days to 56 years). Three cases of cancer were found (SIR, 1.0; 95% confidence interval, 0.20-2.8). One was lymphoma in small intestine, 1 was leukemia, and 1 carcinoma of the uterus but no cases of esophageal cancer. Conclusions Our study is able to exclude long-term risk for esophageal cancer after repair of EA 500-fold higher than that of the normal population. Considering the relatively young age of the survivors, further studies and continued follow-up are warranted to elucidate risk for esophageal cancer and need for endoscopic surveillance after repair of EA.