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Epithelioid leiomyosarcoma (EL) is a rare malignant tumor of mesenchymal origin. The Authors review the literature and report a case of gingival epithelioid leiomyosarcoma in a 40-year-old patient. In this case the leiomyosarcoma was located in the lower front dental group and invaded the symphysis menti. A segmentary mandibolectomy was performed with reconstruction using a non-revascularized autologous iliac bone graft. The differential diagnosis of primary EL is quite complex and it is grouped with other sarcomas, sarcomatoid carcinoma, myoepithelioma, amelanotic melanoma and metastases from gastrointestinal EL. Anatomopathological examination and immunohistochemical study enabled a definitive diagnosis of primary EL of gingiva. The follow-up calls for clinical-radiological check-ups every three months for the first year and every six months thereafter. One year after surgery there were no signs of recurrence.