Neuroendocrine neoplasms of the larynx have either an epithelial or a neural basis. The former are more numerous and are classified as typical or atypical carcinoids and small cell neuroendocrine carcinomas. Paraganglioma is the sole type of neural neuroendocrine neoplasm. There is a significant worsening of prognosis from typical carcinoid to small cell neuroendocrine carcinoma, with the latter having a dismal 5-year survival rate regardless of therapy. Paragangliomas are the most benign of laryngeal neuroendocrine neoplasms, but their clinical behavior may not be predictable on the basis of their histologic appearance.