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OBJECTIVE: To analyze the response and toxicity of updated carboplatin and vincristine (CV) chemotherapy regimen in pediatric low-grade gliomas (LGGs) of the brain stem. METHODS: We modified the traditional CV regimen by increasing the carboplatin dose or combining with a new antiangiogenetic drug named recombinant human endostatin (rh-ES). 10 children who had low-grade gliomas (LGGs) of the brain stem and received modified CV regimen from August 2014 to August 2016 were reviewed. Carboplatin was administrated at a dose of 200~300 mg/m(2) and vincristine was 1.5 mg/m(2) (maximum dose of 2 mg). Three cases out of ten received modified carboplatin and vincristine combining rh-ES. RESULTS: 5 patients had objective response (1 had a complete response and 4 got a partial response). 2 got a minor response and 3 got stable diseases. All the patients’ diseases were effectively controlled. The median time for response was 3 (1.5-8) months. Two of three recurrent children had an objective response. All the three cases with carboplatin, vincristine and rh-ES chemotherapy regimen had objective response. Hematologic toxicity was common. The incidence of severe neutrocytopenia, decreased hemoglobin and thrombocytopenia was 8.8%, 5.3% and 1.5%, respectively. CONCLUSION: Modified CV regimen by increasing carboplatin dose or combining with a new antiangiogenetic drug of rh-ES could improve the effect of CV regimen, and would be helpful to quickly relieve the brain stem dysfunction by shortening the time for response in the pediatric newly diagnosed or recurrent brain stem LGGs. These modified regimens were relatively well tolerated.