Fewer than 20 patients born with situs inversus and duodenal atresia have been reported in the literature. We present a patient with this condition. A newborn baby presented shortly after birth with persistent bilious vomiting. An abdominal radiograph showed a right-sided stomach bubble and a second bubble on the left - typical of duodenal atresia but with mirror image configuration. Laparotomy confirmed the diagnosis of situs inversus abdominalis, which was also demonstrated by contrast studies and ultrasound. Duodenoduodenostomy was performed and the patient discharged on day 8 postoperatively. Situs inversus is associated with other congenital malformations including splenic malformations, left-sided liver and cardiac abnormalities; it is rarely associated with duodenal atresia. Duodenal obstruction in the presence of situs inversus has been described, including obstruction due to a web, stenosis, pre-duodenal portal vein and complete atresia. The patient presented in this paper had a duodenal web in the second part of the duodenum. Before undertaking surgery it is important to establish the presence of associated gastrointestinal and cardiac abnormalities.