Hepatocellular carcinoma (HCC) with extension or metastasis to the right atrium is an uncommon form of cardiac malignancy. This report describes a rare case of right intraventricular metastasis from HCC in a patient who had undergone a partial hepatectomy for HCC more than two years earlier. Open heart surgery was performed and the mass was partially excised. Symptoms (dyspnea, edema of the lower extremities, palpitations) improved after surgery. Two months later a recurrence of the ventricular mass was observed. Systemic chemotherapy with cisplatin and doxorubicin resulted in significant tumor reduction. The patient died of progressive hepatic failure six months later. In addition to this case report, a review of the literature on heart involvement from HCC is presented.