Dementia in old age is usually due to Alzheimer’s disease, cerebrovascular disease, or mixed pathology. Dementia due to other neurological disorders is uncommon, but important to recognize because management may be very different to that in primary or vascular dementia. This chapter surveys five neurological conditions that may present with dementia in later life: idiopathic normal pressure hydrocephalus (INPH); Huntington’s disease (HD); multiple sclerosis (MS); autoimmune limbic encephalitis (LE); and prion disease. For each disorder it reviews the epidemiology, clinical features, investigations, and treatment, with examples of the characteristic brain imaging changes. Accurate diagnosis of these conditions can be challenging even for physicians with a special interest in dementia, and often requires a neurological referral.