Prominent psychotic symptoms, particularly hallucinations and delusions, have been estimated to occur in approximately 60% of patients with dementia with Lewy bodies (DLB). Many of these individuals experience psychotic symptoms before the onset of other features characteristic of DLB. To facilitate consistent diagnosis of DLB, an international consortium recently created consensus guidelines for the clinical and pathological diagnosis of this disease. Accurate diagnosis of DLB is particularly important as these patients may have greater neuroleptic sensitivity than other elderly patients. Currently there is no verified treatment specifically for psychotic symptoms in DLB. However, if neuroleptic treatment cannot be avoided, low doses of atypical antipsychotics may prove beneficial, while minimizing anticholinergic and extrapyramidal side effects.