The authors report a case of IgG4-related hypertrophic pachymeningitis that involved cerebral parenchyma. The mass was removed surgically. Histopathological studies showed diffuse infiltration of lymphoplasmacytic cells without evidence of Langerhans histiocytes or meningothelial cells. Immunoglobulin G4 was strongly positive on immunohistochemical staining. The Gd-enhanced lesion deep inside brain parenchyma was completely resolved after 3 months of oral corticosteroid medication. A nodular type of hypertrophic pachymeningitis that mimics a meningioma is rare. Nevertheless, preoperative presumption is very important, and immunohistochemical studies for IgG4 may be helpful in the differential diagnosis.
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