Cerebral amyloid angiopathy (CAA) is a cerebral small vessel disease caused by the deposition of beta-amyloid in the small- and medium-sized blood vessels of the cerebral cortex and leptomeninges, leading to intracranial vascular amyloidosis. Herein, we report a case of CAA -related inflammation (CAA-ri), a rare clinical condition, focusing on the clinical manifestations, imaging characteristics, cerebrospinal fluid (CSF) findings, and treatment strategies. Key clinical manifestations included psychiatric abnormalities, cognitive impairment, and epilepsy. Brain magnetic resonance imaging revealed asymmetric white matter lesions, whereas susceptibility-weighted imaging demonstrated multiple microbleeds. CSF analysis indicated elevated total protein levels. Following corticosteroid pulse therapy, there was a marked improvement in both clinical symptoms and imaging findings. Given its rarity in clinical practice, early recognition and timely intervention of CAA-ri are crucial for optimizing patient outcomes and enhancing quality of life.