Anatomically corrected malposition of the great arteries is defined as a trunco-conal malformation characterized by an aorta arising anteriorly from a morphologically left ventricle and by the presence of a subaortic muscular conus. This malalignment determines a conotruncal-ventricular discordance: d-loop and l-position or l-loop and d-position of the aorta. Two additional cases of this very rate lesion are reported, both associated with left juxtaposition of the atrial appendages, tricuspid atresia, ventricular malrotation and destrocardia in situs solitus with d-loop. Because of the high frequency of this association, observed also in the majority of the previously published cases, its morphogenetic significance and implications are discussed.