Primary retroperitoneal liposarcoma is a rare low-grade malignant tumor and accounts for approximately 1% of all adult malignancies. Complete gross resection is the most important and maybe only method to cure retroperitoneal liposarcoma. The addition of advanced- modality radiotherapy to surgery for primary retroperitoneal liposarcoma is associated with improved local recurrence-free survival, and the toxic effect is tolerable. Several new drugs, especially targeted drugs, have achieved good efficacy. The aim of this article is to improve the understanding of treatment of primary retroperitoneal liposarcoma. The multidisciplinary therapy model, including surgery, radiotherapy, chemotherapy and targeted therapy, is recommended for patients with retroperitoneal liposarcoma.