INTRODUCTION: Dementia is one of the most important neurological disorders in the elderly. Dementia of tumoral origin is rare and parkinsonism of neoplastic origin is unusual. We herein report a case of gliomatosis cerebri, a very rare brain tumor seldom affecting the elderly, which presented as rapidly progressive dementia and parkinsonism. CASE PRESENTATION: An 82-year-old woman very rapidly developed progressive dementia and akineto-rigid parkinsonism. Brain CT scan was normal. Cerebral magnetic resonance imaging (MRI) with gadolinium injection highlighted a diffuse tumor-related infiltration involving both lobes, the putamen, the pallidum, the substantia nigra, and the brainstem, corresponding to the specific description and definition of gliomatosis cerebri. CONCLUSION: This atypical presentation of a gliomatosis cerebri, and the infiltration of the substantia nigra by the tumor, merits attention.