Objective To explore the clinicopathological features of pediatric-type diffuse low-grade glioma （DLGG） with BRAF V600E mutation. Methods From December 2021 to June 2023， 15 patients with pediatric-type DLGG who underwent surgery at the Department of Neurosurgery of Beijing Tiantan Hospital affiliated with Capital Medical University and were diagnosed with BRAF V600E mutation through postoperative pathology were selected as the study subject. Diagnosis was based on the WHO 5th edition classification of tumors of the central nervous system. The general information of the patient was analyzed， and the histopathological and molecular pathological features of the tumor were explored using hematoxylin and eosin （HE） and immunohistochemical staining， as well as next-generation sequencing. Results Among the 15 patients， 2 were ≤ 6 years old， 9 were 7 to 14 years old， and 4 were ≥ 15 years old. Six were females and 9 were males. Five cases of tumors were located above the cerebellar tentorium， and 10 cases were located below the cerebellar tentorium. Four cases had no clinical symptoms， 6 cases had clinical symptoms of headache and nausea， 3 cases had epileptic seizures， and 2 cases had visual and auditory abnormalities. The next-generation sequencing results showed that all 15 patients had BRAF V600E mutations in their tumors， and gene mutations in the IDH gene and histone H3 were excluded. The combination of next-generation sequencing and immunohistochemical staining ruled out homozygous deletion of CDKN2A/2B. Morphological analysis showed that 13 cases of tumors exhibited the morphological features of diffuse astrocytoma， while 2 cases showed oligodendrocyte astrocytoma morphology. Conclusions BRAF V600E mutation in pediatric-type DLGG is relatively rare， mainly affecting children and young adults， and often needs to be distinguished from other types of intracranial gliomas. In clinical practice， it is necessary to combine microscopic morphological features with patient clinical manifestations and molecular pathological features for comprehensive diagnosis.