The authors present a case-report of a female patient suffering from cerebellar-olivar atrophy, and dementia. Dementia is usually found in heredofamiliar cerebellar degenerative disorders where a cortical parietal and frontal atrophy of the brain lobes is present as well. The authors established a probable diagnosis of the Holmes’ type of the cerebellar-olivar atrophy based on the patient’s clinical state. Only post-mortem autopsy can confirm the diagnosis. The authors consider degenerative cerebellar disorders with proved concurrentdementia in the diferentiation diagnostics.The results of brain imaging correspond with the clinical state of the patient, the results of the psychiatric, neurological, and psychological examinations, and each other. Two main areas of brain impairment are described – cerebellar and parietal. Inspite of a generally poor prognosis of the disease, it is possible to achieve a transient improved clinical condition of the patient by a combination of pharmacotherapy, psychotherapy, and rehabilitation techniques. This is apparent from the presented case.

        Key words: dementia, ataxia, cerebellar-olivar atrophy, imaging methods