Rosai-Dorfman disease (RDD) is an uncommon non-Langerhans cell histiocytosis whose etiology is poorly understood. Recent studies demonstrated that one-third of the patients harbor gene mutations involving the MAPK/ERK pathway. It is considered a self-limited benign process in the majority of cases, with a broad spectrum of clinical manifestations, with patients usually demonstrating massive, bilateral, painless cervical lymphadenopathy. Adolescents and young adults are the most commonly affected, and males are slightly more affected. Extranodal presentation is present in approximately 43% of the cases, usually affecting the eyes, soft tissues of the head and neck, skin, and bone. In the head and neck regions the nasal cavity and paranasal sinuses are the most affected locations. Oral manifestations are also known, including cases affecting the oral mucosa, mandible and maxilla. The presence of clear to foamy histiocytes in a lymphoid background and the occurrence of emperipolesis are very suggestive of the diagnosis, which can be confirmed with S100 and CD68 expression and lack of CD1a and CD207 proteins. There is currently no standardized treatment for RDD, ranging from the watch-and-wait approach to surgery to the use of steroids or chemotherapy.