Neuroendocrine tumors of the larynx represent a rare group of neoplasms characterized by pathological and biological heterogeneity. The histological diagnosis is the most important step in the appropriate management of these tumors and the prognosis varies according to histological types. Here we report on a case of a laryngeal neuroendocrine tumor occurring in a 67-year-old man who underwent several locoregional and systemic treatments. Because of the very low incidence of neuroendocrine tumors in the larynx, a review of the literature has also been performed.