A one-stage total repair was successfully accomplished in a Japanese girl, 7 years, 8 months old, who had asplenia syndrome with vixceral heterotaxia, isolated levocardia, and complicated cardiovascular anomalies; double-outlet right ventricle with d-malposition of the great arteries (anterior aorta, posterior pulmonary trunk), complete form of endocardial cushion defect, total anomalous pulmonary venous drainage, pulmonary stenosis, sinus venosus type of atrial septal defect, patent ductus arteriosus, bilateral superior venae cavae, and inferior vena cava draining into the left-sided atrium. A routine cardiopulmonary bypass, consisting of ligations of the patent ductus arteriosus and right superior vena cava, was followed by direct and patch closure of atrial and ventricular septal defects. An internal conduit was inserted to drain the systemic venous blood from the left ventricle to the pulmonary artery. Thus a total repair is physiologically possible in such complicated cardiovascular anomalies with an asplenia syndrome. One year after this operation the child is well and attends school daily.