Intra-axial pseudotumors in the central nervous system often mimic malignant brain tumors and cause difficulty in diagnosis and treatment. The present study investigates their radiologic and histological features to elucidate diagnostic clues. Six cases were included in the study, one man and five women, ranging in age from 44 to 87 years (mean age, 61 years). Histologically, three cases had demyelination, and one case each had abscess, angiitis, and non-Langerhans cell histiocytosis. All cases were evaluated radiologically on MRI, most of them by thallim-201 single photon emission tomography ((201)Tl-SPECT). These cases were examined using H&E, special stains, and immunohistochemical studies with a variety of antibodies. MRI demonstrated perifocal edema and ring-like or solid enhancement, mimicking the malignant tumors. Diffusion-weighted MRI showed a hypo-iso-intensity with a hyperintensity on the apparent diffusion coefficient. A (201)Tl-SPECT study revealed no uptake. Although there were various kinds of pathology, inflammatory cells were observed, associated with vascular proliferation and reactive astrocytosis. In addition, some cases showed demyelinating or destructive changes. These results suggested that intra-axial pseudotumors in the central nervous system contain various kinds of pathology, and detailed clinicopathological analysis is important from the point of view of differential diagnosis.