A 29-year-old woman with a 4-year history of multifocal fibrosclerosis showed unique neurologic complications. Episcleritis, orbital pseudotumor, and eosinophilic phlegmon preceded intracranial inflammatory pachymeningitis. The pachymeningitis was associated with disturbance of the visual field, incomplete Gerstmann's syndrome, and pseudotumor cerebri. T2-weighted magnetic resonance images revealed a high signal intensity lesion in the left temporal and occipital lobes, and gadolinium-enhanced T1-weighted images revealed the enhancement of the thickened left tentorial leaf. The laboratory data suggested that the etiology might be autoimmunological. The disease and MRI abnormalities improved following administration of corticosteroids.