In order to improve the awareness of cerebral amyloid angiopathy associated with inflammation （CAA-I）， this article reviews the clinical manifestations， imaging features， diagnosis， and treatment of a patient with CAA-I in our hospital. A male patient， aged 70 years， had the clinical manifestations of seizures and cognitive impairment. Cerebrospinal fluid examination showed a slight increase in cell number and protein. Cranial CT showed convex subarachnoid hemorrhage and white matter degeneration； cranial magnetic resonance imaging （MRI） showed abnormal patchy hyperintensity on T2WI and FLAIR adjacent to the cortex， which was asymmetric and extended to subcortical white matter， with the enhancement of leptomeninges； susceptibility-weighted imaging （SWI） showed one or more cortical-subcortical microhemorrhage foci and iron deposition on the surface of the cortex. Genetic testing showed apolipoprotein E E2/E3. The patient was diagnosed most likely with CAA-I. The lesion was significantly improved after methylprednisolone shock therapy， and 1-month follow-up showed improvement in cognitive function without seizure. CAA-I is a rare subtype of cerebral amyloid angiopathy， and there is a lack of understanding of this disease among clinicians. Multi-sequence MRI examination， especially SWI， may help with the diagnosis of this disease， and corticosteroid therapy shows a marked clinical effect in treatment. ［Journal of International Neurology and Neurosurgery, 2022, 49(6): 59-64］