We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the child presented with persistent fever and a diffuse dark red rash. MRI revealed a mass occupying the left nasal cavity and septal sinus and biopsy pathology confirmed the diagnosis of RDD. Following dexamethasone and prednisone treatment, the child experienced dysphagia. 18F-FDG PET/CT revealed multiple lesions with increased metabolism in the left nasal sinus, lymph nodes, widespread skin lesions, and the entire esophagus. Subsequent biopsies of lymph nodes, abdominal skin, and esophageal lesions was consistent with RDD involvement (Fig. 1F-1G). The child is presently undergoing six cycles of VCR+Ara-c+Dex chemotherapy and the treatment is going well.