Hepatocellular carcinoma with extension into the right heart is a recognized, albeit rare occurrence. Patients who present with tumors extending into the heart have generally been considered inoperable and had limited survival, many sustaining tumor embolism and/or sudden death. Resection has been fraught with intraoperative and perioperative mortality as well as considerable postoperative morbidity. We report an exceptional case of a patient with such a tumor successfully treated with an aggressive surgical approach and review the limited published experience.