Portal vein tumor thrombosis is one of the important factors that affect the treatment and prognosis of patients with malignancies. The early diagnosis and treatment of portal vein tumor thrombosis is of great significance to improve the prognosis of patients. However, at present, there is no standard and unified guideline for the diagnosis and treatment of portal vein tumor thrombosis. This article reviews the source as well as the advances in diagnosis and treatment of portal vein tumor thrombosis.