Langerhans' cell histiocytosis presenting as a parotid gland mass is extremely rare. We report a case of Langerhans' cell histiocytosis in the parotid gland that occurred in a 34-year-old Korean male. The patient underwent parotidectomy followed by adjuvant chemotherapy. There has been no evidence of local recurrence or disease progression during 20 months after the lesion was first diagnosed. Differentiation of Langerhans' cell histiocytosis from Kimura's disease was crucial in this clinical setting.
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