Abstract: Hepatocellular carcinoma (HCC)—a common malignant carcinoma in China—has a high mortality rate nationwide. Portal vein tumor thrombosis (PVTT) is a common clinical manifestation and a critical poor prognostic factor of HCC. In patients with HCC and PVTT, liver function and physical status, tumor resectability, PVTT classification, and presence of distant metastasis are important considerations during treatment decisions. Domestic guidelines and expert consensus recommend surgery for patients with partial HCC and PVTT, while most other patients mainly receive systematic therapy, interventional therapy, radiotherapy, and other palliative treatments. Recently, many clinical studies and explorations have been conducted on perioperative treatments for patients with resectable HCC and PVTT and palliative and translational treatments for patients with unresectable HCC. The clinical application of multidisciplinary modalities for patients with HCC is also increasingly advocated and promoted. This study reviews the progress in the treatment of HCC with PVTT.