Dermoid cysts arising from the head and neck region are rare, slow-growing, and well-circumscribed neoplasms. Symptoms are non-specific and usually related to the size and the location of the lesion. A dermoid cyst of the epiglottis is extremely rare. To the best of our knowledge, only one case has been previously reported in the English literature. In this study, a case of a dermoid cyst arising from the epiglottis was presented as an extremely rare clinical condition. The characteristics, differential diagnosis, and treatment of the disease are summarized.