Abstract:ObjectiveThrough the report of a case of Rosai Dorfman disease (RDD), combined with review of literature, the clinical and pathological characteristics, and the diagnosis and treatment methods were analyzed to improve the recognition level of clinicians.MethodsOne patient with RDD admitted to the First Affiliated Hospital of China Medical University in October 2017. Under the guidance of skull base navigation, the patient underwent "Combined Endoscopic Transnasal and Transoral Excision of nasopharynx tumor". After one month of hormone treatment, in January 2019, the tumor of the tongue root was found again and "resection of the tumor of the tongue root" was performed.ResultsThe operation of the patient was successful, and the pathological diagnosis confirmed the postoperative tumors for RDD. The local wound healed well, and no bleeding, pharyngeal stenosis and other complications. The patient was followed up for 1 year without recurrence.ConclusionsPathology is the gold standard for the diagnosis of RDD, and the clinical manifestations and imaging examination of RDD are not specific. For patients with airway obstruction and compression of important parts, surgical treatment should be carried out as early as possible, and hormone can be supplemented after operation. The patient’s regular followup is needed.