We herein report a rare case of pleomorphic lipoma of the tongue with a review of world literature. A 44-year-old woman presented with a nodule of the tongue that had been present for over three years. Clinical examination revealed a yellowish sub-mucosal lesion, measuring 3 cm in maximum diameter, protruding from lingual surface. A first biopsy showed a lipomatous tumour composed of mature adipocytes intermingled with myxoid areas composed of spindle uniform in size and shape and multinucleated floret-like giant cells. Spindle and giant cells were positive for CD34. A diagnosis of pleomorphic lipoma was made. In view of the benign nature of this mass, it was de-bulked rather than completely excised in order to preserve swallowing function.