Neuroendocrine neoplasms (NENs) of the head and neck (H&N) region include a heterogeneous group of neoplastic proliferations arising in the nasal cavity, paranasal sinuses, nasopharynx, larynx, salivary glands, middle ear, and skin. In addition to epithelial neoplasms, H&N paraganglioma and olfactory neuroblastoma can be included in this group. The morphological and clinical features of H&N NENs depend on several different factors, including their degree of differentiation, site of origin, and molecular background. Indeed, H&N NENs encompass a wide spectrum of neoplasms, ranging from indolent neuroendocrine tumors to highly aggressive neuroendocrine carcinomas.