The case of a 16-mth-old patient who had double outlet right ventricle, l-loop and l-malposition in the presence of dextrocardia and situs inversus is presented. A successful repair was performed using an internal conduit to divert the blood from the left ventricle to the aorta. The outflow tract of the right ventricle was then enlarged with a dacron patch. The anatomic variations of double outlet right ventricle surgically corrected are reviewed.