Dermatofibrosarcoma protuberans[DFSP] is an uncommon fibrohistiocytic tumor characterized by aggressive local invasion and consequent local recurrence rather than distant metastasis. We present a case of a 51-year-old male patient with a typical cutaneous DFSP, which showed local recurrences and distant metastasis to the central nervous system[CNS] and the lung after 15 years from the initial diagnosis. CNS metastatic lesions recurred in 5 months after surgical resection of the first CNS lesion. These metastatic tumors were treated by open surgery and gamma-knife radiosurgery. Despite continuous medication of oral chemotherapeutic agent, the patient died during 1-year follow-up period.