To review the clinical, molecular, and imaging landscape of pediatric supratentorial ependymoma (ST-EP) and outline current management principles guiding surgical and adjuvant treatment decisions.

Synthesis of literature regarding biology, diagnosis, and management of ST-EP, focusing on recently emerging molecular subtypes.

ST-EP is an important and clinically significant intracranial malignancy comprising roughly 30–40% of all ependymoma cases. The 2021 WHO molecular classification stratified ST-EP into ZFTA-fusion, YAP1-fusion, and NEC subtypes, fundamentally altering our understanding of this pathology. ZFTA-fused tumors represent the majority of ST-EP, are seen in older patients and harbor a relatively worse prognosis while YAP1-fused tumors, though rarer, are enriched for younger children and carry a substantially more favorable prognosis. Management of very young children presents a unique therapeutic challenge, balancing disease control against the neurocognitive sequelae of cranial irradiation and aggressive surgery in the developing brain.