Plasma cell granuloma is a tumor-like disease characterized by non-neoplastic polyclonal proliferation of plasma cells and other mononuclear cells. This disease occurs most frequently in the lung and upper respiratory tract, while the involvement of the central nervous system is very rare. A 44-year-old female patient presented with nausea and progressive visual disturbance. Brain magnetic resonance imaging (MRI) revealed the mass along the right tentorium with low signal intensity in the T2 weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) sequence, and an isosignal intensity in T1 weighted image (T1WI), the latter of which was enhanced after administration of gadolinium-diethylenetriamine penta-acetic acid (Gd-DTPA). The thickest portion of the tentorium was partially excised via the combined suboccipital and infratentorial approach. The histopathological examination indicated a diagnosis of plasma cell granuloma. Postoperative steroid therapy was administered for remnant tumor control. Although a follow up MRI scan taken 20 months after the operation showed a slight decrease in tumor size, the lesion had extended to the falx and left frontal convexity along with parenchymal edema at 32 months after the operation and the clinical status was aggravated. The mass was removed from the left frontal convexity. Radiation therapy was given, together with steroid administration.