Since the earliest attempt by Klinger in 1931 to describe the systemic vasculitis subsequently characterized in 1936 by Wegener as Wegener's granulomatosis, this disorder has been reported to present in a number of differing fashions. No previous description relates Wegener's presenting as a seizure disorder, and no reports of MRI of the brain in such patients exists. We relate such a case, along with MRI findings, prior to and after treatment, with a review of neurologic manifestations of the disorder.