Primary neuroendocrine tumors of the head and neck are a very rare entity. Poorly
differentiated neuroendocrine carcinomas are highly aggressive tumors associated with
a high propensity for recurrence, distant metastasis and poor survival rates.
We report on the case of a 50-year-old male patient who initially presented with mild
hearing loss due to middle ear effusion. Clinical workup led to the diagnosis of a
nasopharyngeal large cell neuroendocrine carcinoma. The patient was treated with combined
radiochemotherapy.