Nasopharyngeal cancer (NPC) with mastoid recurrence is extraordinarily rare, and its development is thought to involve the Eustachian tube. We herein report a case of NPC with mastoid recurrence masquerading as acute otomastoiditis with facial paralysis in a 60-year-old man 44 months after concurrent chemoradiotherapy. The diagnosis was confirmed by exploratory tympanomastoidectomy with biopsy and Epstein–Barr-encoding region (EBER) in situ hybridization. Distant liver metastasis was detected simultaneously, and the patient underwent salvage treatment.
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