Leiomyoma is a benign tumor that originates from the smooth muscle. If often develops in the uterus and in organs rich of smooth muscle like bowel and subcutaneous tissue. It is infrequently observed in the oral nasal cavities or in paranasal sinuses. The authors, after an accurate revision of the literature, present their first case of zygomatic leiomyoma. They underline the diagnostic aspects in relationship to histologic and immunohistochemical examinations, for surgical therapy.