Neuropathologic confirmation of dementia with Lewy bodies (DLB) involves labeling cytoplasmic Lewy body inclusions for α-synuclein in cortical and subcortical neurons. The authors studied the postmortem brain of a 78-year-old man who had a diagnosis of DLB by exclusion. The patient had symptoms ascribed to DLB that included fluctuating cognitive changes in attention and executive function with progression to dementia, visual hallucinations, and parkinsonism. Sections from the olfactory bulbs and cortical and subcortical regions were stained with periodic acid-Schiff, as well as immunolabeled with antibodies specific for α-synuclein, tau protein, β-amyloid 1-42, and Chlamydia pneumoniae. Most regions demonstrated mixed neuropathologic features, and α-synuclein was notable in Lewy bodies in the amygdala and hippocampus. Periodic acid-Schiff–positive staining was noted in bodies in the amygdala and olfactory bulbs. In this case of DLB, neuropathologic inclusions were consistent with the disease diagnosis, but also with Alzheimer disease and other neurodegenerative diseases, such as polyglucosan body disease.