INTRODUCTION: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a very rare manifestation of cerebral amyloid angiopathy, characterized by acute/subacute neurological deterioration and T2/FLAIR corticosubcortical or deep white matter hyperintensity. With the advent of new diagnostic criteria, there are more and more case reports and series reported; nevertheless, MRI findings and follow-up data need to be thoroughly described.