Cerebral amyloid angiopathy (CAA) is an age-dependent disease affecting older subjects. CAA is characterized by lobar intracerebral hemorrhage (ICH), lobar cerebral microbleeds (CMBs), nontraumatic subarachnoid hemorrhage, and cortical superficial siderosis (cSS), which is the main causes of spontaneous intracranial hemorrhage in the elderly. If a patient had experienced dementia, psychiatric symptoms, recurrent or multiple lobar hemorrhage, the possibility of CAA should be considered. Epilepsy can be associated with CAA. Literature studies had found that CAA-related inflammation are predisposing factors for the development of epilepsy. It is a unique subtype of CAA, which is a form of inflammation and a rare clinical manifestation of sporadic CAA. CAA-ri is a special type of central nervous system vasculitis. Once CAA patients had exhibited atypical clinical manifestations, such as headache, epilepsy, behavioral changes, focal neurological signs, consciousness impairment combined with asymmetric T2 weighted magnetic resonance imaging high signal lesions, clinicians had to consider it maybe CAA-ri. Although CAA- ri is rare, timely diagnosis is important because once seizure had occured, which may indicated the inflammation in CAA patients may had reached a very serious level. Therefore, timely identification and treatment are particularly important. Literature shows that most patients responded well to immunosuppressants. Because of its uncommon, researches on epilepsy in CAA mainly focused on case reports currently, and there were many controversies about its pathological mechanism, treatment and prognosis. This article mainly reviews the incidence rate , pathological mechanism, treatment and prognosis of epilepsy in CAA.