A case of a 24-year-old woman with a 6 weeks lasting nodule of the right margin of the tongue is described. The nodule was 20 mm in diameter and showed surface ulceration. The diagnosis of low grade myofibroblastic sarcoma was supported by histological, immunohistochemical and electronmicroscopic examination. Although the tumor resection was not complete, the patient is free of disease 1 year after operation. The differential diagnostics of low grade myofibroblastic sarcoma is discussed.