Background:Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin [1-3] that rarely presents in the vulva [1,2]. It typically presents on the trunk of young to middle-aged adults[4]. Even though it has a low potential for distant metastases, it often recurs locally [5]. Histopathologically, DFSP typically consists of spindle cells in a “storiform” pattern and stain positive for CD34, which can aid in establishing a diagnosis [1,6-8]. The traditional treatment of choice has been wide surgical excision, but multiple resections are often required for clearance of the lesion [9]. Mohs micrographic surgery (MMS) has recently been described as an alternative treatment option to help decrease the rate of recurrence. Objective: To present a case of dermatofibrosarcoma protuberans of vulva in a young patient treated with excisional surgery with a systematic review of the condition. Case:This patient is a 35-year-old female, G0P0, from The Republic of the Congo (Central Africa), who presented with an eight-year history of a “large left keloid of the mons pubis”. The patient stated the mass was non-tender and the size of a ping-pong ball that changed in size. The patient underwent surgical excision of the “large left keloid of mons pubis”. The specimen consisted of a 6 x 3 x 3 cm elevated portion of smooth tan skin with subcutaneous tissue. It was determined that the specimen was composed of extensive, ill-defined, firm, smooth, homogeneous tan tissue and was sent to pathology for further evaluation. The pathological specimen findings were “Dermatofibrosarcoma protuberans with positive margin”. A second surgery was performed to excise the positive margins of DFSP that remained. Review of an intraoperative frozen specimen found no tumor in the tissue margins. The patient had an uneventful postoperative course and was discharged two days after the surgery was performed. Conclusion:Dermatofibrosarcoma protuberans (DFSP) infrequently involves the vulva and should be considered in the differential diagnosis of other spindle cell lesions presenting in this unusual site [10,11]. The role of immunohistochemical staining with CD34 is imperative in establishing the diagnosis. The rate of local recurrence is high, but it rarely shows metastasis [12]. Surgical excision is the treatment of choice and close follow-up to detect recurrence is imperative [1].