Introduction A large hepatocellular carcinoma (HCC) with macrovascular invasion is generally considered to have poor prognosis due to unfavorable tumor biology andsuch patients are relegated to palliative options. This report describes long term survival after surgery in a patient with HCC and tumor thrombus in the rightatrium. Methods Case records of the patient, details of follow up visits and surveillance computed tomography scans performed were reviewed. A brief discussion of thesurgical strategy adopted along with outcome of similar cases in literature is presented. Results A 60 year old man presented with a HCC in segments 4, 5 with extension of tumor into the middle, left hepatic veins, inferior vena cava and right atrium.Patient underwent extraction of the tumor thrombus from the right atrium under cardiopulmonary bypass along with extended left hepatectomy. He receivedthree cycles of adjuvant chemotherapy with Gemcitabine, Oxaliplatin and Interferon. There was a parietal wall recurrence after 1 year and 10 months whichwas excised. Since then he remains well and is on regular follow up for more than 12 years from his index surgery with no evidence of disease, making himthe longest known survivor with such an advanced presentation. Conclusion This is an unusual instance where aggressive resection for HCC with right atrial tumor thrombus has resulted in an exceptionally long survival.