95 Ependymoma, a rare type of brain tumour, has undergone a transformative understanding over the last two decades, thanks to advancements in molecular research. Historically a challenging condition for paediatric and adult neuro-oncologists, this tumour, originating from ependymal cells in the central nervous system, has traditionally been treated through surgery and radiotherapy. However, outcomes have remained inconsistent, prompting researchers to delve deeper into its biology. The findings were published in the Journal of Neuro-Oncology. The World Health Organization (WHO) has played a pivotal role in advancing the classification of ependymomas. Initial grading systems relied on histological features, but inconsistencies led to the integration of molecular profiling in its 2016 and 2021 updates. These efforts have helped identify 10 distinct subgroups of ependymomas based on molecular markers and anatomical location. Supratentorial, posterior fossa, and spinal ependymomas now represent the primary compartments, each exhibiting unique genetic drivers and clinical behaviours. In the supratentorial compartment, molecular subgroups include ZFTA-RELA fusions, which dominate this category, and YAP1 fusions, primarily seen in infants. ZFTA-RELA fusions have shown a complex relationship with survival outcomes, but recent studies suggest improved prognoses compared to earlier reports. By contrast, posterior fossa ependymomas—subdivided into groups PFA and PFB—display distinct clinical characteristics. PFA tumours typically affect infants and have poor outcomes, especially when associated with specific genetic changes, such as chromosome 1q gain. Meanwhile, PFB tumours generally occur in adolescents and young adults and are associated with a more favourable prognosis. Treatment approaches for ependymomas have largely remained centred around surgery and radiotherapy. Maximal surgical resection continues to be the cornerstone, with focal radiotherapy frequently employed for disease control. Emerging techniques, such as proton radiotherapy, aim to reduce long-term side effects. However, chemotherapy’s role in managing these tumours remains under investigation, with no definitive survival advantage demonstrated thus far. Recent research has shifted attention towards molecularly targeted therapies. For instance, studies exploring the potential of manipulating metabolic pathways, such as with metformin, have shown promise in laboratory settings. Clinical trials investigating agents like 5-fluorouracil and epigenetic therapies are also underway, providing hope for future treatment paradigms tailored to the tumour’s molecular profile. Additionally, immunotherapy approaches, including the use of CAR-T cells, are being investigated to address high-risk subtypes such as PFA tumours with specific genetic abnormalities. While advances in molecular understanding have undoubtedly improved the classification and risk stratification of ependymomas, significant challenges remain. High-risk subgroups, such as PFA tumours with chromosome 6q loss or spinal ependymomas with MYCN amplification, continue to exhibit poor outcomes. These findings underline the urgency for innovative therapies that target the genetic and epigenetic mechanisms underpinning tumour progression. Researchers remain optimistic that these emerging insights will pave the way for more personalised and effective treatments. By bridging molecular biology with clinical practice, the hope is to reduce treatment-related side effects while improving survival rates for patients across all age groups.