We report a case of moderately differentiated neuroendocrine carcinoma arising in the left pyriform sinus and arytenoid of a 68-year-old man. The tumor was diagnosed initially as a poorly differentiated squamous carcinoma, but after surgery it gave rise to multiple painful skin metastases and a paraneoplastic syndrome. Immunocytochemical staining yielded the correct diagnosis. The patient, who had microscopic cervical lymph node metastases at the initial resection, died 13 months later, thus confirming the poor prognosis of these tumors.