This study evaluated survival outcomes of patients with intramedullary Grade II ependymomas and identify prognostic factors. Electronic searches of PubMed, EMBASE, OVID, the Cochrane Central Register of Controlled Trials were performed to identify trials according to the Cochrane Collaboration guidelines. The objects were intramedullary Grade II ependymoma according to 2007 WHO classification. Kaplan-Meier survival analysis with log-rank test was used to analyze progressive free survival (PFS) and overall survival (OS). Cox proportional hazard model was utilized for multivariate analysis with hazard ratio (HR) and 95% confidence interval (CI) calculated. P values <0.05 were considered statistically significant. A total of 28 studies including 138 cases of intramedullary Grade II ependymomas were retrieved. Patients who were classified as cellular ependymomas or papillary ependymomas had higher risks of progression than those who possessed typical Grade II ependymomas. Patients who were treated with adjuvant therapy had a higher risk of progression than those without adjuvant therapy. OS of patients with giant cell ependymoma was significantly shorter than those with typical Grade II ependymoma. Patients who had cellular or papillary subtype, adjuvant therapy would have a shorter estimated value of progression-free time and a higher risk of progression than typical Grade II ependymomas. Giant cell ependymoma patients would have a higher risk of fatality than those with typical Grade II ependymomas. Definite pathology type and appropriate treatments were foundations of intramedullary Grade II ependymomas' managements. (C) 2018 Elsevier Ltd. All rights reserved.