We report a case of intracranial lymphomatoid granulomatosis shown by magnetic resonance (MR) as a focal periventricular lesion. The patient had an undulant clinical course manifested by multiple peripheral and cranial neuropathies. Both the clinical course and the MR findings were difficult to differentiate from an unusual presentation of multiple sclerosis. Autopsy correlation was obtained after the patient succumbed to cardiopulmonary arrest during status epilepticus.