Laboratory tests revealed marked eosinophilia and strongly positive myeloperoxidase (MPO)-ANCA, while electrophysiological studies demonstrated asymmetric axonal neuropathy. A multidisciplinary assessment by neurology and rheumatology confirmed the diagnosis of EGPA with vasculitic neuropathy, following which treatment with corticosteroids, cyclophosphamide, plasmapheresis, and rituximab resulted in significant clinical improvement. This evidence concerns the gene MPO and eosinophilic granulomatosis with polyangiitis.