Background Pediatric high-grade gliomas (PHGGs) consist of a heterogeneous class of central nervous system (CNS) neoplasms with a poor prognosis. We aimed to present our 10-year experience in the management of children with high-grade glioma focusing on patients’ survival and related factors. Methods All pediatric patients with high- grade glioma (HGG) who were admitted to our center between May 2009 and May 2018 were investigated. Overall survival (OS) was calculated from the time of diagnosis until the day of death. The impact of suggested variables on survival was evaluated using the univariate and multivariate analyses. Results There were 41 children with non–brain stem high-grade glioma (NBSHGG). The mean OS of patients was 21.24 ± 10.16 months. The extent of resection (p = 0.002, hazard ratio [HR] = 4.84), the grade of the tumor (p = 0.017, HR = 4.36), and temozolomide (TMZ) therapy (p = 0.038, HR = 3.57) were the independent predictors of OS in children with NBSHGG. Age, gender, tumor location, and size of tumor were not associated with the survival of these patients. Conclusion HGGs are uncommon pediatric tumors with an aggressive nature and a poor prognosis. Our results revealed that in NBSHGG cases, children with maximal safe tumor resection and children that received temozolomide therapy as well as children with grade III of the tumor had higher survival.