Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare disease. Cutaneous RDD (CRDD) is an extremely rare form of RDD, which is limited to the skin. The present study examined a case of purely CRDD in a 25-year-old female patient who presented with a two-month history of red plaques on her face. In addition, a review of the literature was conducted, where the etiology, pathology, clinical characteristics and treatment of the disease were discussed. From a dermatological perspective, the current study aimed to emphasize the histological features and clinical morphology of cutaneous RDD. Clinicians should have sufficient knowledge to be able to recognize and manage this rare condition. The present study found that the presence of reddish-yellow nodules on the face without any particular sensitivity may be useful in the diagnosis of CRDD. Treatment with topical steroids was found to be beneficial in alleviating CRDD.