Neuroacanthocytosis – phenotypically and genetically heterogeneous disorders associated with acanthocytosis – are a group of abnormalities which affect the basal ganglion, causing movement disorders, other neurological symptoms and also cognitive and neuropsychiatric impairments. There are few reports on neuropsychiatric and neuropsychological symptom development in the course of the disease. We described a 33-year-old female patient, who was diagnosed with choreaacanthocytosis associated with neurological, psychiatric and neuropsychological disorders. The neurological disorders included increasing involuntary movements of face and tongue, upper limb chorea, dysarthric speech, slight upper limb deep reflexes, vivid knee jerks, tonic-clonic seizures and absence of Achilles reflexes. Psychiatrically, she was depressed and presented with obsessive thinking. Neuropsychological assessment revealed increasing dysfunctions of attention, immediate memory, learning, verbal fluency, praxia, calculia and difficulties in adjusting behaviour to environmental conditions and in flexible correction of wrong responses. Neuropsychiatric and neuropsychological dysfunctions should be considered in the differential diagnosis to ensure proper diagnosis and management, especially when differentiating disorders in patients with neuropsychiatric symptoms, chorea, or in the case of late (in adulthood) onset of Tourette’s syndrome.