Hepatocellular carcinoma (HCC) with a tumor thrombus (TT) extending into the right atrium is generally regarded as a terminal-stage condition. We report a case of long-term survival following treatment of this complication with en bloc hepatectomy and resection of the thrombus under cardiopulmonary bypass. Our review of 19 similar cases reported in the literature found the following: that lung metastasis, the most critical prognostic factor, occurred in only 5 (27.8%) patients; that postoperative survival ranged from 18 days to 56 months, with a median survival of 11 months; and that 7 (38.9%) patients showed no signs of recurrence, with 4 (21.1%) surviving longer than 2 years. Thus, to prevent sudden death and extend the survival of patients with HCC and TT extending into the right atrium, we advocate simultaneous en bloc resection performed under cardiopulmonary bypass, provided distant metastasis and recurrence in the remnant liver are controlled.