Neuroendocrine neoplasms of the ear are uncommon primary tumors. There is a reported debate in the literature as to the true nature of these tumors and how to classify them. However, there is a kind of consensus that they can represent a spectrum of a common entity with dual glandular and neuroendocrine differentiation. The differential diagnosis of these tumors is broad but their morphologic, immunohistochemical, and radiographic features help to differentiate them from other entities. The tumors have mostly bland histology and indolent biological behavior, yet they may recur and metastasize. It is important to accurately recognize these tumors in order to perform the right management and limit their morbidity. In this article, we review the pathogenesis, histology, immunohistochemical features of neurornedocrine neoplasms of the ear and we discuss their differential diagnosis, clinical behavior and management.