Small cell neuroendocrine carcinoma of the head and neck is rare, and diagnosis may be difficult. Malignancy is high, as is the incidence of distant metastasis. We reported eight cases of stage IV small cell neuroendocrine carcinoma of the head and neck, all in men with a mean onset age of 62 years (range: 45 to 80 years). Three cases arose from the maxillary sinus, two from the ethmoid sinus, one from the parotid gland, one from the tonsil, and one from the larynx. Histological analysis by hematoxylin-eosin staining tentatively revealed malignant lymphoma and undifferentiated carcinoma in two cases each, while immunohistological and/or electron microscopy analysis confirmed histological diagnosis. All were treated by chemotherapy (VP-16, CDDP) and seven cases with radiotherapy based on the schedule of small cell carcinoma of the lung and two cases with lesional resection. Chemotherapy and radiotherapy were effective locally. Five patients died of distant metastasis to the brain, bone, lung, liver, or skin within 12 months. One is alive with liver metastasis. Two have no evidence of disease--one for eight years and the other for one year. Long-term survival thus requires the effective treatment of distant metastasis.