Granulomatous angiitis is a rare, treatable central nervous system vasculitis. Prompt diagnosis may be thwarted by protean presenting symptoms, an indolent clinical course, and atypical neurological findings. The authors describe a case of indolent granulomatous angiitis in which the patient presented with cerebellar signs and tissue changes suggestive of an atypical cerebellar infarction. After several years of remissions and relapses, repeat evaluation and biopsy disclosed granulomatous angiitis both in remote infarctions and in new cortical lesions. The clinical course and neuroradiological and pathological findings are compared with previous reports of fulminant and indolent granulomatous angiitis.