Among the benign pleomorphic adenomas of the minor salivary glands, those that develop in the larynx are very rare. One such case of benign pleomorphic adenoma of the larynx in an adult female is described. The patient was treated with epiglottectomy with a lateral pharyngotomy approach. Review of the literature showed an additional 20 well-described cases of benign pleomorphic adenoma of the larynx. These patients usually present with hoarseness, dyspnea or dysphagia, or any combination of these depending on the site of origin and the size of the tumor. Occasionally the tumor may be discovered incidentally. The tumor can occur in any part of the larynx with the epiglottis being the most common site. Clinically they are indistinguishable from other benign laryngeal neoplasms, as surface mucosal lining remains intact. Histopathological examination is used to make the definitive diagnosis, but rarely, misinterpretation may occur. Conservative surgery provides a lasting cure.