Rosai-Dorfman disease (RDD) is a rare and self-limiting non-Langherhans histiocytosis with lymph node and cutaneous manifestations. This sinus histiocytosis is very extremely usual in children that progress to massive lymphadenopathy. Emperipolesis is a common Histopathological findings and protein S-100 is positive in most cases of RDD. Here, report a case of a 48-year-old with adult with RDD that exhibited cervical lymphadenopathy without fever or Osteolyticlesions. Histopathological and immunohistochemistry studies of lymph node showed emperipolesis, S-100 protein, CD68 positive and macrophages negative for CD1a. After that the diagnosis of RDD was done. However, cervical lymphadenopathy was not solved spontaneously, and the treatment with corticosteroid therapy was initiated and the follow-up showed a clinical improvement condition of the patient.
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