Background: Hepatocellular carcinoma with right atrial tumor thrombus is uncommon but with a dismal prognosis. Methods: By comprehensive literature retrieval of 2000–2019, 53 reports were obtained with 187 patients recruited into this study. The extracted data included patient characteristics, tumor characteristics, treatment, follow-up and outcomes. Statistical analyses applied were student t, Fisher exact and I2 tests. Patients were divided into 6 groups according to the treatment of choices: transarterial chemoembolization (TACE), surgery, radiotherapy, chemotherapy, intervention and supportive care. Results: The overall survival rate of this cohort was 40.8%. The survival rate of patients receiving TACE was 33.3% and that of surgical patients was 41.9%. The survival time of patients with TACE treatment was longer than surgical patients, but lack of statistical significance. Patients were under a follow-up of 15.7±16.6 (median 10) months. Patients receiving radiotherapy had under a longest follow-up among all groups. Intra- and/or extrahepatic recurrence of hepatocellular carcinoma was the major morbidity and also often causes of death. The mortality rates of patients with different treatments in a decremental sequence were supportive care >radiotherapy >surgery >TACE >intervention. No difference was noted in mortality between patients reported from case reports and those from non-case reports. Conclusions: Even though advanced hepatocellular carcinoma with right atrial thrombus is an aggressive malignancy, the present study showed that patients’ prognoses were improved and survival time elongated with active treatments such as TACE and surgery. Active treatments were thus advised to patients with hepatocellular carcinoma with right atrial tumor thrombus.