Systemic necrotizing vasculitis (SNV) is a type of vasculitis that presents with necrosis, predominantly involving large, medium-sized and small arteries. Peripheral neuropathy is a major clinical feature of the primary and secondary systemic vasculitides, and is often observed during the early phases of the disease, causing axonal neuropathy. The prevalence of central nervous system (CNS) involvement ranges from 4% to 45%. Encephalopathy, focal neurological deficits, and seizures are the most common manifestations and usually occur late during the course of SNV. In this report, we describe a 61-year-old woman with SNV who had both CNS and peripheral nervous system vasculitic involvement. We also discuss the pathophysiology of nervous system involvement in patients with SNV.