Delusional misidentification symptoms (DMS) are common in Alzheimer’s disease (AD) and they are frequent sources of serious distress for patients and particularly caregivers. We observed DMS in around 30% of the patients with moderate to severe AD in two independent prospective studies; the Capgras type, phantom boarder, mirror and TV DMS were found most frequently. Patients with DMS showed increased EEG delta-power over the right hemisphere, their CT scans showed more severe right frontal lobe atrophy, and the number of their pyramidal cells in area CA1 was lower than in the patients without DMS. This may indicate that the development of DMS in AD can be promoted by certain patterns of brain degeneration which affect systems relevant to the recognition and updating of memories, while verbal skills may initially be left largely intact.