Dementia with Lewy bodies (DLB) accounts for 10–25% of all dementia cases in clinical populations and is considered to be the second most common degenerative dementia in elderly people after Alzheimer's disease (AD). Dementia with Lewy bodies is characterized by the presence of cognitive, psychiatric, and motor symptoms. Although the neuropsychological profiles of patients with DLB often differ from those of patients with AD, the diagnostic sensitivity, specificity, and predictive values of these profiles remain largely unknown. The present paper reviews the neuropsychological profiling of DLB and attempts the neuropsychological differentiation of DLB from AD.