Aim:Meningiomas, arising from arachnoid meningothelial cells, constitute a substantial percentage of central nervous system tumors. Xanthomatous meningioma, a rare variant marked by lipid accumulation, poses challenges in understanding its epidemiology, pathogenesis, and imaging characteristics. This study presents a systematic review and a case report, aiming to contribute valuable insights into this neoplasm. Material and Methods:The guiding question focused on the epidemiological characteristics of xanthomatous meningiomas. Inclusion criteria encompassed case reports or series detailing patient sex, age, and tumor location. Data collection from eligible studies included patient demographics, symptoms, MRI characteristics, and immunohistochemical markers. Statistical analysis employed SPSS software for nominal qualitative and quantitative variables. Results: The case report involved a 44-year-old female presenting with disorientation and chronic headache, diagnosed with xanthomatous meningioma. The systematic review incorporated 21 studies and 27 patients, with a female predominance and an average age of 48.2 years. Most tumors were located in the frontal region . Common symptoms included headache and seizures . Recurrence occurred in only 7.6% of cases, emphasizing the favorable prognosis post-surgery. Conclusion:This study contributes with valuable insights into the epidemiology, clinical presentation, and imaging characteristics of xanthomatous meningiomas.