In up to 72% of patients with systemic vasculitis the neurological system may be involved. This may be peripheral or central (brain or spinal cord), and is usually due to infarction or a ruptured aneurysm. Diagnosis and evaluation are based peripherally on examination of a biopsy specimen or centrally on angiography. Therapy is usually with steroids and/or immunosuppressive agents. Two patients with systemic necrotizing vasculitis with unusual neurological complications are presented.