-Previous reports on the use of olanzapine in Huntington's disease (HD) used doses ranging from 10-30 mg. We report a case of HD with marked delusions and behavioral impairment assessed by the Unified Huntington's Disease Rating Scale at baseline and four months later treated with a low dose of olanzapine. The patient improved in motor, psychiatric and activity of daily living symptoms after four months of treatment. The response to a low dose of olanzapine in HD may be an indicator of efficacy in similar cases. Further randomized controlled trials can properly assess these findings.KEY WORDS: Huntington's disease, olanzapine, behavioral abnormalities, functional capacity.Resposta funcional e motora a doses baixas de olanzapina na doença de Huntington: relato de caso RESUMO -Relatos de casos sobre o uso de olanzapina na doença de Huntington (DH) usaram doses variando de 10-30 mg. Este é um relato de caso de DH avaliado pela Unified Huntington Rating Scale no iní-cio e quatro meses depois com uma dose baixa de olanzapina. A paciente melhorou dos sintomas motores, psiquiátricos e nas atividades de vida diária após os quatro meses de tratamento. A resposta a baixas doses de olanzapina na DH pode ser um indicador de eficácia em casos similares. Mais estudos controlados randomizados podem avaliar apropriadamente esses achados. PALAVRAS-CHAVE: doença de huntington, olanzapina, transtornos de comportamento, capacidade funcional.Huntington's disease (HD) is a heredodegenerative disorder of the central nervous system, transmitted as dominant autosomic inheritance with a 100% penetrance. It is caused by a three nucleotide basis (CAG) repetition at the IT15 gene, which resides in the short arm of chromosome 4 [1][2] . HD is characterized by insidious onset of neurological manifestations including choreic movements, dysarthria, dysphagia, ideomotor and eyelid apraxia, postural instability, dystonia, dysfunction of eye movements and rarely myoclonus. Incontinence of urinary and anal sphincters (common in the terminal stage of HD), autonomic dysfunction including hyperhydrosis, and pressure lability are other signs and symptoms of the disease. Seizures may occur in 3% of adult HD patients. Neuropsychiatric signs and symptoms may occur before, during or after neurological manifestations. They are present in 35 to 73%, and a wide range of disturbances are reported: affective, behavioral, personality changes which include depression, bipolar disorder, manic episodes, agitation, impulsiveness, aggressiveness. Cognitive symptoms are present in all HD patients at some stage of the disease, characterized by a subcortical dementia profile 3 . The neuroimage shows atrophy of caudate nuclei as the typical feature. The genetic testing is able to confirm the presence of the disease and polymerase chain reaction (PCR) shows an expanded trinucleotide (CAG) repeat sequence.