INTRODUCTION: During repair for duodenal atresia, it has been emphasized that inspection of the small bowel to identify a second atresia is required. The laparoscopic approach for repair of duodenal atresia has been criticized for its limitation to perform this step. Given that duodenal atresia and jejunoileal atresias do not share common embryologic origins, we question the validity of this concern. Therefore, we conducted a multicenter retrospective review of duodenal atresia patients to quantify the incidence of jejunoileal atresia in this population. METHODS: After institutional review board approval (IRB #07-12-187X), a retrospective review was conducted on all patients who have undergone duodenal atresia repair at seven institutions over the past 7-12 years. Demographics and the presence or absence of a jejunoileal atresia were recorded. RESULTS: Four hundred eight patients with duodenal atresia were identified. The mean gestaational age was 36.3 +/- 2.9 weeks, and the mean weight was 2.5 +/- 0.8 kg. Mean age at operation was 19 days (range, 1-1314). There was a 28% incidence of trisomy 21. Two patients (0.5%) were identified as having a second intestinal atresia, and both were type IIIb. One patient was diagnosed at the time of duodenal atresia repair; the other was a delayed diagnosis. Both patients did well after repair. CONCLUSIONS: In this, the largest series of duodenal atresia patients compiled to date, the rate of a concomitant jejunoileal atresia is less than 1%. This low incidence is not high enough to mandate extensive inspection of the entire bowel in these patients, and a second atresia should not be a concern during laparoscopic repair of duodenal atresia.