Lymphomatoid granulomatosis is an angiocentric lymphoproliferative process that involves the lungs. In a 52-year-old-man with lymphomatoid granulomatosis who presented with encephalopathy, magnetic resonance imaging (MRI) of the brain demonstrated unusual multiple areas of enhancement that were both punctate and linear. These findings may be relatively specific for inflammation of deep cerebral vessels and have implications for MRI findings in other inflammatory cerebrovascular disorders.