Objective To clarify the differences of clinical manifestations in patients of Churg Strauss syndrome （CSS） with or without antineutrophil cytoplasmic antibody （ANCA） positive，and primarily explore the roles of ANCA and eosinophilia in CSS according to current literatures. Methods The clinical data of 25 patients with CSS，admitted to Peking Union Medical College Hospital from January 2001 to November 2010，were retrospectively investigated. Results The positive rate of ANCA in Churg Strauss was 32.0%，with P-ANCA predominantly （in 6 patients）. The lung involvement in the patients between ANCA positive and ANCA negative patients was 16.7% and 82.4% respectively with significant difference. The heart involvement in the patients between ANCA positive and ANCA negative patients were 60.0% and 64.2%，respectively.The peripheral neuropathy between ANCA positive and ANCA negative patients were 85.7% and 66.7%，respectively.The renal manifestations between ANCA positive and ANCA negative patients were 75.0% and 35.3%，respectively. But the differences in heart，peripheral neuropathy，orrenal manifestations were not significant. Conclusion The status of ANCA may represent different clinical phenotype of CSS.