Cerebral amyloid angiopathy (CAA) is the result of the deposition of an amyloidogenic protein in cortical and leptomeningeal vessels. The most common type of CAA is caused by amyloid beta-protein (Abeta), which is particularly associated with Alzheimer's disease (AD). Excessive Abeta-CAA formation can be caused by several mutations in the Abeta precursor protein and presenilin genes. The origin of Abeta in CAA is likely to be neuronal, although cerebrovascular cells or the circulation cannot be excluded as a source. Despite the apparent similarity, the pathogenesis of CAA appears to differ from that of senile plaques in several aspects, including the mechanism of Abeta-induced cellular toxicity, the extent of inflammatory reaction and the role of oxidative stress. Therefore, therapeutic strategies for AD should, at least in part, also target CAA. Moreover, CAA and cerebrovascular disease (CVD) may set a lower threshold for AD-like changes to cause dementia and may even cause dementia on its own, since patients with AD and CAA and/or CVD appear to be more cognitively impaired than patients with only AD. In conclusion, the precise impact of CAA on AD or dementia remains unclear, however, its role may have been underestimated in the past, and more extensive studies of in vitro and in vivo models for CAA will be needed to elucidate the importance of CAA-specific approaches in designing intervention strategies for AD.