Abstract
Ependymomas are uncommon tumors arising from ependymal cells of the central nervous system, with lipomatous metaplasia representing an extremely rare pathological variant. We report a case of a 28-year-old male who presented with a 5-year history of an incidentally discovered intracranial mass and recent-onset dizziness. Magnetic resonance imaging (MRI) revealed a left frontotemporal lesion characterized by significant fat signal, progressive enhancement, and mass effect on adjacent structures. The patient underwent gross-total resection, and histopathology confirmed a World Health Organization (WHO) Grade II ependymoma with lipomatous metaplasia. Immunohistochemical analysis further supported the diagnosis, highlighting features of both ependymal differentiation and fatty metaplasia. We also provide a comprehensive literature review of previously reported cases, emphasizing the rarity, imaging characteristics, differential diagnosis, and surgical considerations for this unique pathological variant. This case underscores the importance of careful radiological and pathological evaluation to guide diagnosis and management, and aims to raise awareness of this rare subtype among clinicians and pathologists.