Approximately 20% to 40% of dementia is caused by diseases other than Alzheimer's disease. This article reviews the major categories of non-Alzheimer dementia, including dementia associated with cerebrovascular disease, dementia associated with extrapyramidal features, and the frontotemporal dementias. Dementia associated with cerebrovascular disease is a heterogeneous condition the importance of which is often misunderstood. Dementia with Lewy bodies, the most common of the dementias associated with extrapyramidal disease, is becoming better recognized for its unique management issues. At least some of the frontotemporal dementias, which in this article encompass the progressive aphasias, have mutations in the tau gene that account for some of the phenotypic variations.