Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that frequently recurs locally, but rarely metastasizes. The purpose of this work is to present a clinical series of DFSP patients and national Danish incidence data in the period 2000-2012. Furthermore, the aim is to present guidelines on the management based on a review of the literature. Medical records of 26 consecutively treated patients at the Department of Plastic Surgery in Health Care Region Zealand were reviewed and histological specimens were reassessed. To investigate national Danish incidence in the period 2000-2012, data were extracted from the national pathology registry. Finally, a literature search was performed in Pubmed and Cochrane, and 23 major publications were reviewed. Studies on Mohs Micrographic surgery were excluded. All patients were treated with wide local excision (WLE) with a median margin of 2.8 cem and a median follow-up time of 36 months. We found a local recurrence rate of 4%. Our national incidence data were based on 374 patients. The overall incidence was 0.53 per 100,000 persons. The prevalence of DFSP in the age group 20-50 years was significantly higher than the group below 20 years (p < 0.0001). Surgery is the treatment of choice for primary DFSP, local recurrences, and metastases. If clear margins cannot be obtained by WLE or surgery is not an option because of unacceptable functional or cosmetic outcome, adjuvant radiotherapy or imatinib can be considered. Chemotherapy can be a final option if other treatments fail.