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Pleomorphic xanthoastrocytoma (PXA) is a rare glioma that affects 1% of astrocytic tumors. It most commonly affects children and teenagers. PXA cases with an anaplastic histopathological subtype have been reported in recent years. It was recently classified as a separate subtype in the 2016 World Health Organization (WHO) classification of central nervous system (CNS) tumors. A 48-year-old healthy gentleman presented with progressive right upper limb weakness. CT and MRI of the brain were done, which showed an intra-axial supratentorial tumor. A diagnosis of high-grade glioma was initially made based on its imaging features. The histopathological study came back as anaplastic pleomorphic xanthoastrocytoma. After a discussion with the neurosurgical and oncology teams, a decision was made to treat the patient with radiotherapy. In this case report, we describe a rare case of PXA with anaplastic …