目的提高对窦组织增生症伴巨大淋巴结病(Rosai-Dorfman病)的临床表现和病理诊断的认识。方法报道1例经淋巴结活检确诊Rosai-Dorfman病患者的临床资料并结合文献进行讨论。结果患者肺CT平扫示双侧肺野内可见多发、弥漫性分布的大小不等的结节影,纵隔内可见多组肿大淋巴结影。淋巴结病理可见组织细胞、浆细胞和淋巴细胞并可见吞噬淋巴细胞现象,免疫组化S-100及CD68阳性。该病临床表现多样,易误诊。结论 Rosai-Dorfman病累及气道是罕见的淋巴结外病变,治疗以综合治疗为主,治疗后随访。 Objective To improve the understanding of the clinical manifestations and pathological diagnosis of sinus hyperplasia with giant lymphadenopathy (Rosai-Dorfman’s disease). Methods A case of Rosai-Dorfman disease diagnosed by lymph node biopsy was reported and discussed in the literature. Results The CT scan of the lung showed multiple nodules in the bilateral lung fields with diffusely distributed nodules of various sizes. Multiple sets of enlarged lymph nodes were seen in the mediastinum. Pathological lymph nodes visible tissue cells, plasma cells and lymphocytes and phagocytosis of lymphocytes seen, immunohistochemical S-100 and CD68 positive. The clinical manifestations of the disease is diverse, easily misdiagnosed. Conclusion Rosai-Dorfman’s disease is a rare extranodal disease involving the airway. The treatment is mainly based on comprehensive treatment and is followed up after treatment.