Clinical data of 15 primary central nervous system lymphoma(PCNSL)children aged≤18 years admitted to our hospital between May 2013 to May 2023 were retrospectively analyzed.Our goal was to summarize the clinical features of children and investigate the therapeutic effect of a high-dose methotrexate(HD-MTX)based chemotherapy regimen on this disease.The male-to-female ratio was 2.7:1,and the median age was 7.2(2.3-16.4)years at diagnosis.The initial clinical symptoms were primarily cranial hypertension,with imaging findings revealing multiple lesions.Pediatric PCNSL with normal immune function has a favorable prognosis with HD-MTX-based chemotherapy.Patients with a stable disease can be treated with minimal or no maintenance.HD-MTX-based chemotherapy remains effective when the disease progresses or recurs after an initial course of non-HD-MTX-based chemotherapy.