To evaluate the pathological features, clinical manifestations, diagnosis, treatments and prognosis of Rosai-Dorfman disease (RDD) in head and neck.

HE staining and immunohistochemical analysis were performed in a case with both nodal and extranodal RDD in head and neck, follow-up data was collected and pertinent literatures were reviewed.

Characteristic histiocytes with abundant pale or eosinophilic cytoplasm, and many lymphocytes and plasma cells were observed under light microscopy. A condition termed emperipolesis was found. Immunohistochemical staining was positive for both S-100 and CD68 but negative for CD1a. The clinical manifestation, along with destruction and resorption in maxillofacial bone, was atypical in this case and therefore apt to make a misdiagnosis. The patient shows partial remission on regular clinical and radiological follow-up followed immunotherapy.

Although RDD in head and neck has its specific pathological features, it presents differential clinical presentations and is hard to make an accurate diagnosis. Various treatments were used but none of them proved to be widely effective. Conservative treatment may be suitable for RDD in head and neck, and moreover, a long-term follow-up is indispensable.