Dermatofibrosarcoma protuberans is a rare neoplasm, usually presenting as a low-grade, non-invasive, slow-growing tumor, with a pronounced tendency to local recurrence. We present a rare case of a high-grade, histologically undifferentiated lesion, which eroded underlying bony structures. A wide local excision with careful pathologic analysis of margins was performed with immediate reconstruction of skull bone and musculo-cutaneous flap transposition in order to cover the skin defect. A one-stage surgical procedure, such as presented in this report, is the treatment of choice recommended by the authors.