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Abstract Central nervous system high‐grade glioma (HGG) occurring in an infratentorial midline location is not commonly found in the pediatric population. Though pediatric HGGs appear similar to their adult counterparts histopathologically, they differ in molecular, genetic, and clinical characteristics. Evidence for the management of HGG is sparse in the literature. Surgery in the form of maximum safe resection is the backbone of management and has been variably supplemented with external beam radiotherapy and cytotoxic chemotherapy. The outcome, though largely dismal, has some positive surprises too. As shown in the presented case, the combined modality of management in a 5‐year‐old female child has resulted in a disease‐free survival of 3.5 years.

Central nervous system high‐grade glioma (HGG) occurring in an infratentorial midline location is not commonly found in the pediatric population. Though pediatric HGGs appear similar to their adult counterparts histopathologically, they differ in molecular, genetic, and clinical characteristics. Evidence for the management of HGG is sparse in the literature. Surgery in the form of maximum safe resection is the backbone of management and has been variably supplemented with external beam radiotherapy and cytotoxic chemotherapy. The outcome, though largely dismal, has some positive surprises too. As shown in the presented case, the combined modality of management in a 5‐year‐old female child has resulted in a disease‐free survival of 3.5 years. [ABSTRACT FROM AUTHOR]