A case of primary myxoid leiomyosarcoma of the ovary in a 50-year-old Tunisian woman is presented. Bilateral salpingooophorectomy and hysterectomy were carried out without any adjuvant therapy. The tumour were investigated histologically and immunohistochemically. Smooth-muscle actin and progesterone receptors was strongly demonstrated in neoplastic cells, bcl-2 was weakly and diffusely demonstrated. Relevant literature is reviewed based on the histologic and immunohistochemical features with emphasis on diagnosis and therapeutic problems and prognosis indicators.