Lewy body disease is a common cause of neurodegenerative dementia. The clinical syndrome of dementia with Lewy bodies (DLB) has specific diagnostic, care and management needs, is underrecognized in clinical practice, and often mistaken for Alzheimer’s disease. It may only be identified at autopsy. It typically has a worse prognosis after diagnosis than Alzheimer’s disease. Early, accurate identification is important; improving this at clinical and prodromal stages may assist effective management and reduce patient and caregiver burden. The characteristic clinical features must be recognized: parkinsonism, RBD, fluctuating attention and cognition, complex visual hallucinations. In clinically equivocal cases, diagnosis may be supported by biomarkers specific to Lewy body disease: dopaminergic imaging, cardiac sympathetic imaging, polysomnography. A pattern of cognitive impairment not typical of Alzheimer’s disease may also be seen, with implications for cognitive symptom screening. Screening may be aided by seeking other symptoms of Lewy body disease (hypolfaction, autonomic symptoms, hallucinations other than visual), and other neuropsychiatric symptoms (delusions and mood changes).