Abstract: A 56-year old female patient with cutaneous Rosai-Dorfman disease (CRDD) was reported and the related literature was reviewed. The only clinical manifestation was nodules and dull red plaques on her right arm for 2 years without any systemic damage. The biopsy showed dense dermal inflammatory infiltrates composed of histiocytes and lymphocytes. Some histiocytes displayed emperipolesis. Immunohistochemical staining revealed that the histiocytes were positive for S-100 and CD68, negative for CD1α. The lesion scope was obviously shrunk after local application with halometasone.