EZH2 is a component of the polycomb repressive complex 2 (PRC2), which is a highly conserved histone methyltransferase that methylates lysine 27 of histone 3. EZH2 mutations are associated with oncogenesis and progression of cancers. However, the relationship between the clinical outcome of patients with myeloid malignancies and EZH2 mutations is controversial. Therefore, we performed a meta-analysis of 8 studies (n=2243 patients) that evaluates the correlation between EZH2 mutations and overall survival (OS) in patients with myeloid neoplasms. EZH2 mutations were associated with significantly worse OS (hazard ratio[HR]Z 2.37, 95% confidential interval (CI), 1.48-3.79). In a word, EZH2 mutations indicate a poor prognosis for patients with myeloid neoplasms.