The clinicopathologic features of two cases of paragangliomas of the larynx are presented along with immunohistochemical, ultrastructural and DNA image analysis. The world literature is also critically reviewed in order to develop a more accurate clinicopathologic profile of the tumor. The results indicate that paragangliomas of the larynx occur in patients averaging 47 years of age and are 3 times more common in women. Most (82%) arise from the supraglottic larynx and manifest clinically as hoarseness. They are typically benign and rarely functional. Almost all alleged malignant paragangliomas of the larynx are in reality atypical carcinoids that have been misdiag-nosed. Features that distinguish paragangliomas from other similar tumors are discussed.