Objective: To review the clinical, morphologic, immunohistochemical, and histogenetic characteristics of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma and explore current treatment options. Methods: We describe the case of a 38- year-old patient with a tumor measuring 5.7 cm on the right labium majus of the vulva. Serial sections stained with hematoxylineosin were examined and immunohistochemical staining was performed for CD34 and PDGF receptor alpha and beta (PDFGRA and PDGFRB). Results: The histologic study showed spindle-cell proliferation typical of dermatofibrosarcoma protuberans and other areas containing fibrosis and giant cells lining pseudovascular spaces. Both tumor areas expressed CD34, PDGFRA, and PDGFRB. Conclusions: Only two cases of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva have been reported to date. Both dermatofibrosarcoma protuberans and giant cell fibroblastoma are characterized by the translocation t (17;22) (q22;q13). The fact that PDGFRA and PDGFRB are overexpressed in these tumors opens new treatment options with imatinib. Surgical excision with wide margins or Mohs micrographic surgery continues to be the treatment of choice.