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Wen Sijian, Sun Jianfang. Department of Pathology,Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China Corresponding author: Sun Jianfang, Email: fangmin5758@aliyun.com ��Abstract�� Dermatofibrosarcoma protuberans ��DFSP�� is a rare borderline malignant or potentially low-grade malignant neoplasm with a high local recurrence rate and a low risk of metastasis. DFSP usually occurs on the trunk in people aged 20-50 years, and is characterized by multiple indurated nodules varying in size and arising on atrophic plaques. Histologically, the tumor consists of uniform spindle cells which grow infiltratively and are arranged in a storiform or cartwheel pattern. The standard therapy of DFSP is wide local excision with a margin of 2 �� 3 cm. Mohs′ surgery and modified Mohs′ surgery have shown a good prospect in the treatment of DFSP. Radiation therapy can serve as an adjuvant treatment after surgery. In recent years, the pathogenesis of DFSP has been further clarified and studies have shown that chromosomes 17 and 22 are rearranged in 90% of patients with DFSP. As a targeted drug, imatinib has been approved for the treatment of DFSP in patients with unresectable, metastatic and/or recurrent DFSP.