This study reports a clinical case of cutaneous Rosai-Dorfman disease, its manifestation, and its evolution in 10 years, thus contributing to the limited bibliography about it and which review is also an important aim of this paper. This clinical study included a histopathological and immunohistochemical review of the lesions. Since this is a skin disease, not a life-threatening condition, and due to the lack of therapeutic resources and funds, only one excision surgery of a small lesion was performed without further therapeutic attempts. This article states the importance of distinguishing this disease from other malignant ones, because of the different prognosis made in each case. Treatment is necessary only for cosmetic purposes or in case of becoming a life-threatening condition. It is also suggested that Rosai-Dorfman disease and its cutaneous form could be two different entities.