Introduction/Background*Low-grade fibromyxoid sarcoma (LGFMS) is a rare and unpredictable soft-tissue fibrous tumor that classically arises in proximal extremities and the trunk.

The occurrence in the female external genitalia is exceptionally described in the literature.

Methodology This report presents two patients with documented LGFMS arising from the vulva who were treated in our institution between 2019 and 2020.

Result(s)*The patients were aged 22 and 31 years respectively.

Both women presented in another department with painless, slow-growing nodule of the vulva evolving for two years in one case and two months in the other case.

Physical examination found a well-defined subcutaneous mass of the right labia majora of 3 cm in the first case and 10 cm in the second case. The tumors had a smooth surface and hard consistency.

Local excision was performed under local anesthesia.

The histological examination revealed a mesenchymal proliferation of spindle cells occupying the dermis and the hypodermis with positive margins.

Immunochemistry confirmed the diagnosis of LGFMS.

The patients were then referred to our institution.

Pelvic MRI performed in one patient revealed a contrast-enhancing epidermal mass of the right posterior part of the vulva measuring 23×13 mm with no local lymphadenopathy.

CT scan showed no distant metastasis in both patients.

After a multidisciplinary consultation meeting, we decided to perform a tumor bed resection.

The final pathologic testing revealed no residual tumor with negative final margins.

No adjuvant treatment was considered necessary to complete the treatment.

The current follow-up of the two patients is free of recurrence.

Conclusion*LGFMS are among the least common histologic subtypes of sarcoma arising in the lower genital tract, and its benign appearance can be misdiagnosing.

Surgery with immunohistological examination remain the only way to establish the diagnosis and guide the treatment.