Peripheral neuropathy is common in many vasculitic syndromes and may be the only manifestation of the underlying vasculitic disease. Although traditional teaching has been that a true multiple mononeuropathy is the classic clinical presentation of vasculitic neuropathy, an overlapping (or extensive) multiple mononeuropathy or a distal symmetric polyneuropathy is commonly encountered. Similarly, the leukocytoclastic reaction has traditionally been considered the primary mechanism of vessel injury in these diseases, although more recent evidence suggests that cellular-mediated mechanisms may be more important in peripheral nerve. In this review, new concepts concerning the clinical presentation, pathogenesis, diagnosis, and treatment of vasculitic neuropathy are discussed, particularly in relation to the syndrome of isolated peripheral nerve vasculitis.