Objective To summarize the updates of diagnosis and differential diagnosis for hepatocellular carcinoma (HCC) with portal vein tumor thrombus (PVTT) for providing evidences for early diagnosis and treatment of PVTT patients. Methods The related literatures on diagnosis and differential diagnosis for HCC with PVTT in recent years were collected and reviewed. Results The serious complications and tumor metastasis are attributed to the PVTT, then it is necessary to make diagnosis accurately according to clinical symptoms, hematological and imaging examinations. The differential diagnosis of PVTT and portal vein thrombosis, portal sponge degeneration and hepatic arteriovenous shunt diseases should be carried out. Conclusions The diagnosis and differential diagnosis of PVTT cannot rely on a single method, and it requires a comprehensive judgment of various diagnostic methods. More accurate and specific diagnostic methods are needed.