We report the case of a malignant primary cardiac pheochromocytoma treated by adjuvant cytotoxic chemotherapy after surgical resection, with a 5-year survival. There is no specific chemotherapy for malignant pheochromocytoma, but because it has the same embryologic origin as neuroblastoma, we used similar chemotherapy. Because of unexpected malignancy potential, we think that total and meticulous resection of the tumor must be done.