BACKGROUND Hepatocellular carcinoma (HCC) accompanied by a tumor thrombus is very common. However, the treatment strategy is controversial and varies by the location of the thrombus. CASE SUMMARY We report herein a case of HCC with a tumor thrombus in the suprahepatic inferior vena cava (IVC), which was successfully treated by hepatectomy combined with thrombectomy following sorafenib chemotherapy. A 47-year-old woman with chronic hepatitis was diagnosed with HCC. Computed tomography and magnetic resonance imaging showed that the tumor lesion was located in the right half of the liver, and a tumor thrombus was detected in the suprahepatic IVC near the right atrium. After multi departmental discussion and patient informed consent, right major hepatectomy and total removal of the tumor thrombus were successfully performed under cardiopulmonary bypass. There were no serious complications after surgery. Following sorafenib treatment, no recurrence has been detected so far (11 mo later). CONCLUSION Surgical treatment followed by adjuvant sorafenib therapy might be an acceptable choice for HCC patients with tumor thrombosis in the IVC.