A case of Creutzfeldt-Jakob disease, Heidenhain's form is reported. The first clinical manifestations were cortical blindness and visual agnosia. The patient here concerned, a woman aged sixty three, during the clinical course of the disease showed mental deterioration and pyramido-extrapyramidal manifestations. She died after five months. The electroencephalographic findings showed periodic activity. The anatomopatological examination showed neuronal degeneration, status spongiosus and proliferation of astroglial cells. The clinical, electroencephalographic, pathological and etiopathogenical aspects are discussed.