We describe one patient with crossed atrioventricular connection in situs inversus, concordant atrioventricular connection, double outlet right ventricle, and straddling of the right atrioventricular valve. Angiographic features are analyzed, pointing out the importance of selective angiography in order to define the atrioventricular and ventriculoarterial connections. The utility of bidimensional echocardiographic study before cardiac catheterization is emphasized in order to know the associated malformations.