An otherwise healthy 20-year-old male presented with an exophytic, polypoid, yellowish lesion involving the dorsal surface of his tongue, which he reported being present since birth and unchanged. This was removed by surgical excision and diagnosed as a leiomyomatous hamartoma. Histological examination revealed a combination of fibrovascular connective tissue, conspicuous smooth-muscle bundles, adipose tissue, minor salivary gland tissue, blood vessels, lymphoid tissue, peripheral nerves, and normal skeletal muscle. This case is exceptional due to the patient’s age, as until now, lingual leiomyomatous hamartomas have been reported almost exclusively in a paediatric population. To our knowledge, this is the eldest age at which a LLH has been reported in the literature. This underscores the need for clinicians to consider this rarely reported entity when considering the radiographic and clinical differential diagnoses for these lesions, both in the paediatric and adult populations. We also present a review of the literature regarding lingual leiomyomatous hamartomas.