Symmetrical lipomatosis of the tongue is an extremely rare condition. To date, only eight cases have been reported in the literature. We present an extremely rare case of intramuscular lipomatosis of the tongue in a 67-year-old male. The present case is unique because of its infiltrating nature and the extension of the lesion. Glossectomy was performed to reduce the size of the tongue and for diagnosis. The literature is reviewed and clinical characteristics, pathology, and surgical treatment are discussed.
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