Angiomyxolipoma is a rare histological variant of lipoma, characterized by proliferation of adipose tissue associated with a variable amount of myxoid stroma with numerous thick- and thin-walled blood vessels. An exhaustive literature review could reveal only 12 cases of angiomyxolipoma affecting subcutaneous tissue, subungual area and spermatic cord. In oral cavity, only 2 cases of angiomyxolipoma have been reported to date, one case in the buccal mucosa and one case in the floor of the mouth. Hence, this is only the third case of angiomyxolipoma affecting oral cavity and the first case of angiomyxolipoma of the tongue. We report a case of a 51-year male presenting with a swelling on the right lateral border of the tongue. The lesion was excised and microscopical examination confirmed the diagnosis of angiomyxolipoma.