Atypical monoclonal plasma cell hyperplasia, like plasma cell granuloma, is an inflammatory pseudotumor. Both are extremely rare in the central nervous system. Atypical monoclonal plasma cell hyperplasia is a recently identified neuropathological entity described by Weidenheim, et al., in 1989. A second case of this disease entity is now reported. The histological findings that differentiate this lesion from plasma cell granuloma, plasmacytoma, and meningioma are discussed. The present case clearly demonstrates the complete resolution of the disease after a course of fractionated radiotherapy.