ObjectiveTo investigate the clinical manifestations, diagnosis, treatment and prognosis of Rosai-Dorfman disease (RDD) in nasal cavity and nasopharynx. MethodsWe retrospectively analyzed the clinical data of 4 patients with extranodal RDD admitted to the Department of Otolaryngology, Head and Neck Surgery of our hospital between November 2009 and August 2013. ResultsAll patients received complete surgical removal of the lesions. Histopathological examination showed that large histocytes had an abundant foamy cytoplasm containing ingested lymphocytes as a result of emperipolesis, and were strongly positive for S-100 and CD68 on immunohistochemical analysis, but negative for CD1a. All patients were followed up for 28 to 45 months (averaging 35 months), and no recurrence was observed during the follow-up period. ConclusionRDD is a rare disease, and the exact etiology and pathogenesis of RDD are not fully understood. There is no unified treatment plan recommended, and complete surgical removal of the lesions is an effective method. Minimally invasive surgery may be a good choice for treatment of this disease.