The present report describes therapy and follow-up of a patient who experienced a localized swelling of the neck that proved to be a mucoepidermoid carcinoma. Extensive staging revealed no primary site. Therapy was modified neck dissection followed by external application of chemotherapy combined with fractionated radiotherapy. Over a period of 43 months, the patient has remained free of local tumour growth and distant metastasis. Whereas prognosis of mucoepidermoid carcinoma in general is good, prognosis of patients affected by mucoepidermoid carcinoma with carcinoma of unknown primary is ambiguous.
