目的　探讨鼻窦部结外Rosai Dorfman病的临床表现、组织学特征、诊断、鉴别诊断及治疗。方法　分析国内罕见的发生于鼻窦的结外Rosai Dorfman病的临床、组织学和影像学资料。结果　病变位于鼻窦 ,曾先后多次复发、手术切除并病理误诊。组织学分析可见大量梭形细胞混有泡沫细胞和少量浆细胞 ,胞质丰富呈泡沫样。免疫组化分析显示 ,组织细胞呈S 10 0和CD6 8阳性。结论　鼻窦部结外Rosai Dorfman病诊断困难 ,易误诊 ,临床应与鼻硬结病、嗜酸性肉芽肿、浆细胞瘤、良性纤维组织细胞瘤等进行鉴别 ,免疫组化细胞呈S 10 0、CD6 8阳性有助诊断。此病可采用化疗、放疗和手术治疗等综合治疗 ,对重要部位的病变侵犯 ,彻底的手术切除是最有效治疗方法 ,术后应跟踪随访 Objective To investigate the clinical manifestations, histological features, diagnosis, differential diagnosis and treatment of Rosai Dorfman’s disease outside the sinonasal node. Methods We analyzed the clinical, histological and imaging data of Rosai Dorfman’s disease in our country, which is rare in our country. Results lesions in the sinus, has repeatedly relapsed, surgical excision and pathological misdiagnosis. Histological analysis showed a large number of spindle cells mixed with foam cells and a small amount of plasma cells, cytoplasm rich foamy. Immunohistochemical analysis showed that the tissue cells were S 10 0 and CD 6 8 positive. Conclusions Rosai Dorfman’s disease is difficult to diagnose, easily misdiagnosed, clinically diagnosed with rhinosclerosis, eosinophilic granuloma, plasmacytoma, benign fibrous histiocytoma and so on. The immunohistochemical cells are S 10 0, CD 6 8 Positive help diagnosis. The disease can be chemotherapy, radiotherapy and surgical treatment and other comprehensive treatment of lesions of major parts of violations, complete surgical resection is the most effective treatment, follow-up should be followed up