Objective To analyze the clinical features of eosinophilic granulomatosis with polyangiitis (EGPA) and the characteristics in different anti-neutrophil cytoplasm antibody ANCA) state. Methods The clinical characteristics, treatment and outcomes in 31 cases with EGPA who met the EGPA classification criteria of ACR in 1990 from West China Hospital of Sichuan University during the period between January 2011 and September 2020 were collected and retrospectively analyzed. Results Among the 31 patients with EGPA, 64.5% had respiratory symptoms or systemic symptoms as their first symptoms. During the course of disease, 77.4% had asthmatic manifestations, 61.3% had ear, nose and throat involvement, 96.8% had lung infiltration,54.8% had peripheral neuropathy, 29% had central nervous system involvement,54.8% had cardiac abnormalities, 45.2% had skin manifestations,32.3% had renal damage,29% had gastrointestinal tract involvement, and 6.5% had ocular involvement. In terms of laboratory examination, all patients had increased eosinophils in peripheral blood. 91.3% of those who had completed IgE test had increased IgE. ANCA-positive rate was 48.4%, and 80% were MPO positive. Compared with the negative group, the ANCA-positive group was more prone to renal involvement，with higher IgG and PLT, and lower Hb and albumin, with statistically significant differences. However, the ANCA-negative group was more prone to skin or gastrointestinal tract involvement. In the terms of biopsy, 75% showed increased eosinophil infiltration around small vessels, 25% showed pathological features of small vasculitis, and 6.25% showed inflammatory granuloma. According to the severity of the patient's condition and organ involvement, corticosteroids or combined with immunosuppressants and gamma globulin were main therapy. In this group, 83.87% of the patients evaluated by FFS≥1 indicated poor prognosis, while those with FFS=3 were all ANCA-positive EGPA and had very poor prognosis. However, most of the other patients were relieved after treatment, and further follow-up was needed for long-term prognosis. Conclusion EGPA is a rare disease which can involve multiple organs. During the course of EGPA, eosinophils are often increased, and asthma, sinusitis, wandering pulmonary infiltration, and peripheral neuropathy are often presented. The positive rate of ANCA is not high, so attention should be paid to the identification of ANCA-negative EGPA to avoid missed diagnosis. ANCA-positive patients tend to have poor prognosis, so individual assessment should be taken and active treatment should be adopted.