Primary plasma cell leukemia (PPCL) is an uncommon and very aggressive hematological disease, which has different cytogenetic abnormalities, special molecular and biological characteristics, compared with multiple myeloma(MM). The clinical course is unfavorable with short remissions and reduced overall survival(OS). Intensive chemotherapy regimens and bortezomib-based regimens, immunoregulatory drugs(IMiDs) as well as stem cell transplantation may be beneficial to PPCL patients. This review summarizes the recent advances of this malignant disease, including clinical and biochemical characteristics of PPCL, therapy and prognosis of PPCL, new drugs for PPCL and so on.