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Huntington’s disease is an incurable, adult-onset, autosomal dominant inherited disorder associated with cell loss within a specific subset of neurons in the basal ganglia and cortex. Characteristic features of HD include involuntary movements, dementia and behavioral changes such as depression, episodic bouts of mania, psychosis, obsessive-compulsive symptoms, sexual and sleep disorders and changes in personality. The aim of this case report is to discuss diagnostic and therapeutic challenges in a patient with a mutation in the gene responsible for the development of Huntington’s disease (HD), who presented schizophrenia-like psychotic symptoms. A 53-year-old, married, secondary school graduate, unemployed, male with genetically-confirmed HD for 12 years was hospitalized to our psychiatry service because of aggression, self-mutilation, avolition, dysphoric mood, insomnia, disorganized speech and jealousy delusions about his wife. He had movement disorder for nearly 20 years and his psychotic and affective symptoms were occurred 7 years ago, but for one month within this period, all symptoms were increased and difficulty in swallowing had developed. He had already used quetiapine, aripiprazole and benzodiazepine but these treatments enhanced choreic movements in our patient. After the hospitalization we organized his treatment as olanzapine 20 mg/day IM, clonazepam 3 mg/day. Especially his psychotic symptoms declined but because of swallowing problem persisted and he was transferred to neurology service. Patients with HD can exhibit various psychopathological symptoms. Before the diagnosis of HD, patients may also apply to us with psychiatric symptoms. Therefore, we consider that, it was important know the different types of psychiatric aspects of the illness for the diagnosis and treatment.