Dermatofibrosarcoma protuberans is a rare cutaneous tumor with particular characteristics and a high frequency of recurrence after inadequate primary treatment. Its histopathological diagnosis might be difficult. Dermatofibrosarcoma protuberans can be safely distinguished from other similar neoplasms of mesenchymal origin based on the immunohistochemical expression of CD34 antigen and the genetic presence of specific chromosomal translocations. Although rarely metastatic, it is followed by a significantly high rate of locoregional failure due to an indolent subcuticular tissue spread. Aggressive surgical management is the therapeutic approach of choice. A wide resection with microscopically disease-free margins is always recommended. Mohs' micrographic surgery together with advanced reconstructive techniques provides satisfactory results even for tumors involving the face or distal extremities.