Duodenal atresia (DA) is a congenital malformation characterized by failure to recanalize the portion of the small intestine that extends from the pylorus to the angle of Treitz (duodenum). Although AD represents up to 60% of cases of intestinal atresia, its incidence associated with situs-inversus is an even rarer case, with only approximately 20 cases described in the literature. Even so, an early diagnosis and approach are necessary in order to promote the clearance of the gastrointestinal tract and, consequently, improve the patient's quality and life expectancy.