The association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial communication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital cardiac malformations has not previously been reported in patients with isomeric left atrial appendages.