A four-year-old boy who had isolated levocardia associated with left atrial isomerism, polysplenia and tetralogy of Fallot underwent successful corrective surgery. In addition, these anomalies were complicated by interrupted inferior vena cava, azygos continuation, and total anomalous hepatic venous connection. Because of these systemic venous anomalies, cardiopulmonary bypass presented us some problems of venous cannulations. We used 4 direct venous cannulations; i.e., superior vena cava, persistent left superior vena cava through coronary sinus, azygos vein and hepatic vein. Reports of total correction in patients with isolated levocardia associated with tetralogy of Fallot are rare, and we report the case with some reviews of the related literature.