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¹Sağlık Bilimleri Üniversitesi, Hamidiye Tıp Fakültesi, Haydarpaşa Numune SUAM, Patoloji Bölümü İstanbul, Turkiye Meningiomas are the most common primary central nervous system (CNS) tumors. They are usually benign, slow growing neoplasms that are thought to arise from meningothelial (arachnoid) cells. The World Health Organization (WHO) has a standardized grading system consisting of 3 groups and 13 histological subtypes determined for this tumor group. This grading system is closely related to the risk of recurrence and overall survival and therefore has important implications for treatment strategy. WHO grade I meningiomas account for 80.5% of all meningiomas and have benign histology and silent clinical behavior. Grade II and III meningiomas account for 17.7% and 1.7% of all meningiomas, respectively. Since rhabdoid meningiomas are defined within this classification, they are included in the grade III anaplastic meningiomas group. However, recent studies reveal the biological behavior and genetic characteristics of rhabdoid meningiomas and point out that this rare tumor type should be viewed from a different perspective. In this study, it is aimed to present the data of rhabdoid meningioma by scanning the literature in recent years. Anahtar Kelimeler : Meningioma, Rhabdoid meningioma, BAP1 mutation