Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. The etiology and pathogenesis of RDD have not been fully explained, and its occurrence is even rarer in the maxillofacial region. This paper reported a patient who was first misdiagnosed as facial inflammation caused by oral disease and finally diagnosed as RDD. Clinical features, diagnosis, treatment, and prognosis were discussed based on the literature. This work provides reference for future diagnosis and differentiation of RDD in cheek.