Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, affecting between 10 and 14 cases per million globally. This adult disorder has a mean age at diagnosis of nearly 50 years and affects both sexes equally, presenting with pulmonary symptoms (asthma), eosinophilia and granulomatous or vasculitic involvement of several organs. A multidisciplinary, multinational European group has developed evidence-based, guidelines for the diagnosis and management of EGPA, intended to include recent advances.