Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.