Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis characterized by eosinophil-rich and necrotizing granulomatous inflammation in the peripheral blood and involved organs. According to whether antineutrophil cytoplasmic auto-antibodies (ANCA) can be detected in the serum of the patients or not, EGPA is assigned into ANCA-positive or -negative phenotypes. The ANCA-positive patients are prone to purpura, glomerulonephritis, peripheral neuropathy, and alveolar hemorrhage. Myocardial and lung involvement are more common in those with ANCA-negative. In this article, we reviewed the literatures concerning the differences in the clinical manifestations and involved organs between the ANCA-positive and -negative EGPA, and summarized their clinical characteristics.