The authors describe a case of a 28-year-old patient with Chediak Higashi’s syndrome manifested by a combination of Parkinsonian syndrome, dementia, cerebellar syndrome and immunodeficiency. It is a patient with incompletely expressed signs of the mentioned syndrome and an atypical combination of neurological symptoms as compared with published cases but with a typical morphological finding in the haemogram.

        Key words: Chediak Higashi’s syndrome, adult form, „giant lysozomes“ granulocytes