Advanced hepatocellular carcinoma (HCC) with invasion into the heart through the hepatic vein is a recognized rare occurrence with an extremely poor prognosis. Patients who present with right heart tumor thrombus have generally been considered inoperable. Although aggressive resection and liver transplantation treatment have previously been performed, the results remain unsatisfactory. However, HCC with extension into the heart usually indicates a contraindication for transcatheter arterial chemoembolization (TACE). In this study, a rare case of HCC with metastatic inferior vena cava (IVC) and right atrial (RA) tumor thrombus was reported. The young patient was admitted to our department due to Budd-Chiari syndrome. Following diagnosis according to CT image findings and laboratory data, the patient underwent TACE therapy. This treatment resulted in a marked reduction in the liver tumor and the right atrial tumor thrombus. Following TACE therapy, the patient survived for 3 years and 10 months and remains alive without any signs of recurrence. This case indicates that TACE therapy can be used successfully for the treatment of advanced HCC with heart tumor thrombus and may result in long-term survival.