Abstract
Ependymomas are categorized based on anatomical location and specific genetic alterations, with extra-axial metastasis being a rare event, occurring in less than 1% of cases and documented sparsely in the literature. This case study details a 27-year-old male patient diagnosed with supratentorial ependymoma characterized by Zinc Finger Translocation Associated (ZFTA) fusion and World Health Organization (WHO) Grade 2 morphology. Additionally, a systematic review of all reported cases of extra-axial ependymoma metastases was conducted, systematically compiling clinical, morphological, and molecular data from relevant articles. Metastatic ependymoma represents a rare occurrence characterized by diagnostic and therapeutic challenges. A comprehensive review of the literature could provide valuable insights into the underlying biology and support the selection of optimal treatment strategies for such cases.