Early diagnosis of dementia with Lewy bodies (DLB) – the second most frequent type of primary degenerative dementia following Alzheimer’s disease – is still a tall order in everyday clinical practice. This paper reviews the revised criteria for the clinical diagnosis of DLB and therapeutic options in patient management. Patient management in DLB is a complex and difficult matter, including early detection, performing necessary investigations and finally establishing diagnosis. Treatment, consisting of both nonpharmacologic and pharmacologic interventions, has to be multimodal and targeted at compensation of cognitive impairment, assessment and management of neuropsychiatric and behavioral symptoms, alleviation of parkinsonian symptoms, monitoring and management of autonomic dysfunction and sleep disorders. Cholinesterase inhibitors are the drugs of choice in DLB (evidence favours rivastigmine) and may provide benefit for both cognitive impairment and activities of daily living. The effectiveness of DLB treatment is reported as being generally more pronounced than in Alzheimer’s disease, despite using the same class of medications. In case cholinesterase inhibitors are ineffective it may be necessary to prescribe an atypical antipsychotic. First generation antipsychotics should be avoided and clinicians should be aware of the possibility of a severe neuroleptic hypersensitivity reaction.