Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic eosinophilic vasculitis, belonging to the group of ANCA-associated vasculitides (AAV). EGPA pathogenesis is still elusive, but a potential role for IL-5 and Eotaxins mediated eosinophil activation has been reported. Imatinib mesylate, a tyrosine kinase (TK) inhibitor, is approved for the treatment of chronic myeloid leukaemia and FIP1L1/PDGFRA positive gene fusion hypereosinophilia. A potential role for imatinib in the treatment of idiopathic hypereosinophilic syndrome (HES) and AAV has been recently proposed