Patients with congenital heart disease are increasingly surviving to adulthood and present interesting challenges during cardiac catheterization. Situs inversus with dextrocardia is a type of cardiac malposition most likely to exist with a structurally normal heart. We present the case of a patient with complete situs inversus and bicuspid aortic valve stenosis, a hitherto unreported association. Prototypical findings in this cardiac malposition are illustrated for proper recognition with emphasis on technical tips for left and right heart catheterization.