The purpose of this study is to investigate the patient characteristics and clinical outcomes of children with pediatric brainstem glioma. Between 2004 and 2009, a total of 42 children were diagnosed with brainstem gliomas at the Neurosurgical Center of Beijing Tiantan Hospital, China. A retrospective study including the 33 patients of this cohort with complete follow-up was conducted in an attempt to better understand clinical outcomes following multidisciplinary treatment modalities for pediatric brainstem gliomas. Investigational variables including clinical presentations, anatomical distribution, radiological findings, and clinical outcomes were analyzed. Survival time difference was computed using a Kaplan-Meier method with a log-rank test between groups. The Cox proportional hazards regression model was utilized in the multivariate analysis to determine the independent prognostic factors. Overall median survival of the entire series of patients was 11 months with a 1-year actuarial survival rate of 43.6 %. In nine patients who received no treatment after diagnosis, all patients expired within 8 months with a median time of 3.5 months. On univariate analysis, the following variables including older age at diagnosis, higher Karnofsky Performance Status score at diagnosis, the lower pathological grade, surgical resection modality, increasing diagnostic latency, and focal growth pattern were associated with better survival. On multivariate analysis, only the last two variables were associated with survival advantage. Focal pediatric brainstem gliomas amenable to a surgical resection are likely to achieve a prolonged survival. Clinical trials on larger number of patients are of importance in further understanding this spectrum of devastating diseases.