Purpose: To further improve the effectiveness and prognosis of primary hepatocellular carcinoma (HCC) with portal vein tumor thrombus (PVTT), the current status of treatment for HCC with PVTT was reviewed. Methods: A Medline search was undertaken to identify articles using the keywords "HCC", "PVTT" and "therapy". Additional papers were identified by a manual search of the references from the key articles. Results: PVTT, as a common complication of HCC, was divided into type I similar to IV. The therapeutic approach is mainly composed of five types: surgical resection, regional interventional therapy, radiotherapy, combination therapy, targeted therapy. All of these therapeutic approaches were separately evaluated in detail. Conclusions: For those resectable tumors, the better choice for treatment of HCC with PVTT should be hepatectomy and removal of PVTT. For those unresectable tumors, TACE (especially the super-selective TACE) has been the preferred palliative treatment, the other regional interventional therapy and/or radiotherapy could improve the therapeutic effects. The multidisciplinary treatments may further improve the quality of life and prolong the survival period for the HCC patients associated with PVTT. (C) 2015 Published by Elsevier Ltd on behalf of IJS Publishing Group Limited.