The objective of this work is to describe the neuropathological findings of a patient clinically presenting with rapidly progressive nonspecific neurological symptoms suggestive of Creutzfeldt-Jakob disease. Methods used were clinical description with laboratory analyses, repeated electroencephalogram, cerebral computed tomography, magnetic resonance imaging studies and details on neuropathological work-up. Neuropathological examination excluded Creutzfeldt-Jakob disease. By contrast other neurodegenerative changes combining Alzheimer-type pathology and Lewy body pathology were detected as the most likely substrate of neurological symptoms. Dementia with Lewy bodies should be included in the differential diagnosis in individuals presenting with rapidly progressive dementia.