Paraganglioma and atypical carcinoid tumor of the larynx are two neuroendocrine neoplasms that have often been confused in the past, and even in the present, in the literature. The clinicopathological profile of the two lesions is presented and the differential diagnosis is discussed. A correct diagnosis is of paramount importance, since treatment and prognosis depend on diagnostic accuracy and differ for the two lesions. Paraganglioma of the larynx is usually benign, whereas atypical carcinoid tumor is malignant and has an aggressive clinical course.