Small cell neuroendocrine carcinoma is an unusual laryngeal carcinoma accounting for slightly less than 0.5% of carcinomas. To date, approximately 125 cases have been reported. These tumors most often present in the 6th and 7th decades in men who are heavy cigarette smokers. Patients usually complain of hoarseness and almost 50% present with cervical lymph node metastases. These tumors are biologically aggressive with at least 73% of patients dying with widespread tumor metastases. Two- and 5-year survivals are 16 and 5 %, respectively. Combined radiation and chemotherapy offer the best hope of survival.