Abstract： Objective To improve the recognition of diagnosis, treatment and leukemia transformation of primary myelofibrosis (PMF). Methods One case with PMF which transformed into acute myeloid leukemia (AML) was reported and the literatures on this topic were reviewed. Results The patient was diagnosed according to the 2008 version of the WHO classification of PMF leukemia transformation was diagnosed after 2.5 years. The JAK2 V617F gene mutation was detectable both before and after leukemia transformation. The outcome of low dose chemotherapy could keep a short time. Conclusion JAK2 V617F gene could remain detectable after leukemia transformation in PMF patients. It is difficult to control the disease progress by low dose chemotherapy.