We present a rare case of a rabdoid meningioma and study the clinicopathological characteristics of the neoplasm. Results: Α 68-year-old woman was submitted in surgical excision of an endocranial tumour (d:3 cm) in our hospital. The histological examination revealed entirely rabdoid morphology. The neoplastic cells expressed the following immunohistochemical phenotype: S100+, Vimentin+, EMA+, GFAP−, PR+, HMB45−, A1/AE3+(focally), CK8/18+(focally), SMA−, Ki-67 (<1)%. Conclusion: Rabdoid phenotype represents an indicator of malignant transformation regardless of the tumor's histogenesis. Expression of Ki-67 antigen correlates with recurrence even in meningiomas with minimal atypical features, which should prompt closer surveillance after excision. Rabdoid meningiomas behave aggressively and have bad prognosis contrary to the classic meningioma. Prognosis is influenced by it's complete excision and the expansion within the intracranial cavity. It has to be distinguished from glioma, melanoma and metastatic carcinoma. The patient after 1 year is in generally good health, and is followed up by oncologists.