Creutzfeldt-Jakob disease is a rare neurodegenerative disease that can occur in four forms. The sporadic form, the familial form, the iatrogenic form and variant Creutzfeldt-Jakob disease. The sporadic form is seen in about 1 per million people per year and is associated with rapidly progressive cognitive decline, psychiatric and neurological symptoms. The diagnosis and counseling of patients is a major challenge because of the complex and heterogeneous presentation, especially if the presentation differs from the standard. In this article we describe a diagnostic struggle regarding an elderly patient.