Dementia often is defined as a progressive cognitive disturbance leading to a loss of independent function. Most clinicians are familiar with the typical pattern of amnestic Alzheimer's disease, the most common neurodegenerative presentation of dementia. Atypical dementia presentations, including atypical Alzheimer's variants, however, may pose a diagnostic challenge for even experienced clinicians. In this article the authors discuss clinical “pearls” for the diagnosis of various neurodegenerative dementia syndromes. When considering the causes of dementia, the mnemonic VITAMINS can be helpful in considering various etiologies.