Vulvar sarcomas located in the Bartholin's gland area are extremely uncommon mesenchymal vulvar tumors. These neoplasms can be mistaken as Bartholin' gland benign lesions such as cysts or abscesses, leading to a delay in the diagnosis of underlying malignancy. Currently, only a few cases of these aggressive cancers have been reported in the literature. A 42-year-old female patient without any previous complaint presented to Obstetrics and Gynecology Department of 'G. Chaztikosta' General Hospital due to a vulvar lump in the area of the left Bartholin's gland with a 6-month history of progressive swelling. Pelvic examination showed a solid mass of 6.5-cm in maximum diameter, localized in the left Bartholin's gland. The patient underwent wide local excision and histopathological examination of hematoxylin and eosin-stained sections indicated intersecting fascicles of spindle cells, with moderate to severe atypia. The number of mitoses was up to 8 per 10 high power fields. The neoplasm to its greatest extent was circumscribed and in places had an invasive growth pattern. Tumoral necrosis was not seen. Involved Bartholin' gland by the tumor was identified. The tumor extended focally to the surgical margin. The neoplastic cells showed positive staining for smooth muscle actin, desmin, HHF35, caldesmon, vimentin and estrogen and progesterone receptors. Immunohistochemistry was negative for S100, myoglobulin, keratin 116, CD117, CD34 and CD31. The patient denied further surgery or/and local radiotherapy, although the mass was >5-cm and a focally infiltrative surgical margin was found. During the close follow-up, no local recurrences or metastases were observed 53 months after surgery. In conclusion, wide local tumor excision with free surgical margins is a good option of surgery for vulvar leiomyosarcomas. In recurrences, a new extensive surgical resection of the lesion and radiotherapy are suggested. Ipsilateral lympadenectomy is indicated when there is a pathologic lymph node. Chemotherapy is provided in cases of distal metastases.