Lymphangiomas are rare, congenital lesions of the lymphatic system, and about 90% of them are detected by the 2nd year of life. Although the head and neck region is the most common place of presentation, isolated laryngeal lymphangioma is extremely rare. A 37-year-old female patient presented with characteristic symptoms of acute epiglottitis. After her acute symptoms resolved with medical treatment, endoscopy was performed, and a wide, pedunculated mass arising from the epiglottis of the larynx was seen. The mass was totally excised and microscopically diagnosed as lymphangioma. To our knowledge, this is the first such case reported in the literature. In the control examination performed after 9 months, no evidence of recurrence was revealed. This interesting case illustrates that the symptoms of acute epiglottitis in the adult should be further investigated to exclude rare lesions such as lymphangioma.