Myxoid leiomyosarcoma (MLMS) of the vulva is a mesenchymal tumor with only five reported cases in the literature. We report an 85- year- old woman with a unilateral nonulcerating, painless vulvar mass. According to the patient, the mass slowly enlarged. Initial biopsies were benign. However, clinically, this lesion was suspicious for a soft tissue tumor. Therefore, the mass was removed by a wide local excision. Definitive histology revealed a myxoid leiomyosarcoma of the vulva. At present, 25 months after the operation, the patient is well with no sign of recurrence. Vulvar myxoid leiomyosarcomas are rare and can be confused with other benign or malignant tumors. It is important to be aware of this rare tumor variant, in order to plan appropriate treatment.
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