Proliferative histiocyte disease, rarely found in the dermatovenerologist practice. Rosai-Dorfman disease is a benign disease of unclear etiology, may be accompanied by cervical nodes extensive lymphadenopathy and extranodal lesion. The absence of pathognomonic signs leads to a long-term diagnostic search with diagnosis verification only by histological method. The clinical cases rare description in dermatological practice is an important work aspect and a necessary component of expanding the knowledge and practicing physicians experience. Purpose: to describe the case of the Rosai-Dorfman cutaneous form with differential search. Materials and methods: detailed clinical data analysis, including a symptoms description and changes on the skin, as well as the results of a pathohistological study. The Rosai-Dorfman cutaneous form features and potential pathogenetic mechanisms of the disease are discussed. Currently, there is no specific pathogenetic treatment for the Rosai-Dorfman cutaneous form. The therapy which used was symptomatic and based on the symptoms elimination.