A 68-year-old woman presented with generalized clonic seizure following a 2-month history of initiative loss, incoherent speech, headache, and left hemiparesis. No systemic signs or symptoms were seen and laboratory studies were within normal range. Computed tomography and magnetic resonance imaging demonstrated a well-delineated small mass with homogeneous enhancement in the right parietal convexity, associated with unusually extensive perifocal edema compared to the size of the mass. Cerebral angiography showed a faint stain fed by the middle meningeal artery. These imaging features were very similar to those of meningioma. Full recovery from the symptoms was achieved by total removal of the lesion and no recurrence was found after 3 years. Histological examination identified the hyaline-vascular type of angiofollicular lymph node hyperplasia (Castleman's disease). Castleman's disease involving the central nervous system is rare, with only 12 previous cases, but should be considered in the diagnosis of intracranial meningeal tumors. The treatment of choice for localized Castleman's disease is complete surgical resection, which is curative in most of the cases.