Only few cases of scalp dermatofibrosarcoma protuberans with intracranial and distant metastasis have been reported. Here we report a case of scalp dermatofibrosarcoma protuberans with frequent local recurrence, intracranial invasion and with distant lung metastasis during 6 years of treatment. We would like to emphasize difficulties in surgical treatment of such invasive and locally recurrent tumors of scalp, and necessity to understand new molecular pathogenesis of dermatofibrosarcoma protuberans and potential treatment strategy with imatinib for patients with surgically untreatable disease. Close surveillance of patients with scalp dermatofibrosarcoma is necessary due recurrence nature of tumor.