Background: Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis which predominantly involves the lung, skin, gastrointestinal tract and the nervous system, but here we report a CSS case involving the mediastinal lymph node (LN) and gallbladder (GB). Case report: A 43-year-old woman with 2-year history of asthma was admitted with dyspnea and wheezing. The patient showed marked peripheral eosinophilia and multiple mediastinal lymphadenopathy with multifocal pneumonic consolidations on both lower lungs in chest computed tomography (CT). The patient was treated with methylprednisolone and antibiotics assuming asthma exacerbation with pneumonia. Following 7 days of treatment, dyspnea improved with near disappearance of pulmonary in. ltration. However, during steroid tapering, acute cholecystitis with multiple GB stones developed. Follow-up chest CT showed much improved consolidations, but no significant change of enlarged mediastinal LN. In addition, serum P-anti-neutrophil cytoplasmic antibody was positive. Because she had persistent abdominal pain requiring cholecystectomy, co-operation of cholecystectomy and concurrent wedge resection with mediastinal LN biopsy was performed for tissue confirmation. Necrotizing and granulomatous vasculitis with eosinophil in filtrates was observed in the specimens of GB, lung, and LN, respectively. Furthermore, skin biopsy was performed from purpuric lesions on both dorsum and it showed leukocytoclastic vasculitis with eosinophil predominance. finally, she was con. rmed as CSS based on asthma, eosinophilia, polyneuropathy, pulmonary in filtrates and extravascular eosinophils by biopsy confirmation on multiple sites. After 4 weeks of treatment with methylprednisolone 60mg/day, her associated symptoms and eosinophilia were improved. Conclusion: We herein report an unusual case of CSS manifested by mediastinal lymphadenopathy with lung consolidation and necrotizing eosinophilic cholecystitis, which can be misdiagnosed as pneumonia with reactive LN enlargement and stone induced cholecystitis. Thus, involvement of CSS in other organ should not be overlooked.