Dermatofibrosarcoma protuberans (DFSP) is a rare malignant dermal neoplasm characterized by slow infiltrative growth, little metastatic potential but a high tendency to recur locally after surgical excision. DFSP is associated with a high cure rate. The optimal therapy is complete surgical resection. The recurrence potential of DFSP is directly related to the extent of resection. The need for wide excision margins has been amply documented. Wide local excision is a frequently used practice. Mohs micrographic surgery with continuous histological margin control is further propagated to reduce local recurrence rates. In more than 90% of DFSP, a specific chromosomal aberration is described, involving Chromosomes 17 and 22. It leads to a constitutive activation of the platelet-derived growth factor receptor (PDGFR) followed by continuous stimulation of the tumor cell growth. The use of targeted inhibitors of PDGFR is a good therapeutic option in the treatment strategy of unresectable locally advanced, recurrent or metastatic disease. With Imatinib, a selective PDGFR tyrosin kinase inhibitor, partial and complete remissions of DFSP could be achieved. This article reviews the current opinion and literature about DFSP and resulting therapy strategies.