Granulomatous angiitis is a frequently fatal central nervous system vasculitis of unknown pathogenesis sporadically associated with Hodgkin's disease, mixed cellularity type. We describe a 58-year-old woman presenting with headaches, progressive spastic paraparesis, equivocal computed tomography findings, and magnetic resonance imaging findings of increased signal intensity biparietally on T2-weighted imaging in a relatively discrete pattern. Magnetic resonance imaging-directed serial stereotactic biopsies revealed granulomatous angiitis throughout the right parieto-occipital region and splenium. Simultaneous biopsy of an enlarged submandibular mass revealed Hodgkin's disease, lymphocyte-predominant type. This case shows that granulomatous angiitis may be associated with magnetic resonance imaging findings suggesting a mass lesion in lymphocyte-predominant as well as mixed cellularity Hodgkin's disease.