Non-systemic vasculitic neuropathy is one of many localized vasculitides, with involvement restricted to nerves and (possibly) muscles. Inclusion and exclusion criteria differ between reported cohorts. All require a nerve biopsy diagnostic of or suspicious for vasculitis and no extra-neuromuscular involvement. Patients typically present subacutely with a painful, multifocal/asymmetric, distal-predominant neuropathy. In the absence of clinical or laboratory evidence of systemic vasculitis or a condition predisposing to such, prognosis with treatment is good. Pathological data are supportive of a primary T-cell-mediated immunopathogenesis. Some patients classified as having non-systemic vasculitic neuropathy have a systemic vasculitis presenting with neuropathy; in others, the disease is organ-specific.