Abstract. Huntington’s disease is a hereditary neurodegenerative disease with diagnostic challenges when psychopathological disorders are manifested, despite the typical choreic symptoms. In the present article, we present a clinical case of a sixty year old patient with motor and cognitive disorders, disorganized behavior, paranoid delusions, and a family history of schizophrenia. Both the “pitfalls” misleading the correct diagnosis and the distinguishing features pointing to it are described. In-depth analysis of the clinical symptomology and the course of the disease through generations, data from neuroimaging studies, and genetic testing are all decisive for the diagnostic process. The case is a further evidence of the role of the multidisciplinary approach in overcoming diagnostic “pitfalls”, providing a better understanding of the different clinical variations of Huntington’s disease.

Key words: Huntington’s disease, schizophrenia, cognitive impairment, psychosis

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