In recent years, the cardiovascular manifestations of systemic lupus erythematosus have became more apparent as a consequence of both prolonged survival and improvement in diagnostic modalities. We report the case of a 16-year old woman with systemic lupus erythematosus in whom the presence of cardiomyopathy was characterized by endomyocardial biopsy. This diagnostic technique also showed that the histologic hallmark of lupus myocardiopathy persisted despite corticosteroid and plasmapheresis, suggesting the need of a careful cardiac follow-up in these patients.