We report a case of an infant with isomerism of the right atrial appendages in whom a spleen-like nodule was found at post-mortem. The nodule, on histology, was found to be a fragment of liver. The cardiac lesions were typical of those associated with right isomerism, a complex generally described as the “asplenia syndrome.” The presence of either a spleen, or a nodule masquerading as a spleen, does not preclude an isomeric arrangement of the atrial appendages nor its commonly associated constellation of cardiovascular anomalies.
