The clinical course, pathological features and response to treatment in a consecutive 16-years series of 19 cases of dermatofibrosarcoma protuberans were studied. A review of the world literature, comparing the results to ours too was performed. The tumour, occurring at every age and equally among females and males, most commonly involves the trunk without any particular pattern. The disease usually runs an indolent course and is too often regarded lightly by patients as well as physicians leading to delayed and often too conservative treatment. The tumour has definite invasive and metastatic potential and is considered a low grade soft tissue sarcoma. To avoid mutilating surgery (and maybe metastases) later in the course, the initial treatment must be wide surgical excision including a surrounding margin of at least 1 inch normal tissue. The removal of underlying deep fascia is essential and adequate resection will require a skin graft replacement in nearly every instance. Follow up periods should be no less than 3 years at the surgical and oncological service and should be continued at the general practitioners because tumours may recur after considerable periods of time, even in apparently adequately treated patients.