The term, dermatofibrosarcoma protuberans (DFSP), was coined by Hoffman in 1925 to signify a protuberant, often multinodular cutaneous tumor that showed progressive growth. Darier and Ferrand had described a case of the disease a year before, and in France, the term, sarcome Darier-Ferrand, is still in current usage. Albeit rare, DFSP is the most common tumor consistently regarded as a cutaneous sarcoma (atypical fibroxanthoma is more common, but is not universally thought of as a sarcoma), with an incidence of approximately 4 per million individuals per year in the United States, affecting blacks twice as commonly as whites, and frequently arising on truncal skin. The tumor usually affects middle-aged adults but may also occur in children or the elderly. Its histogenesis remains uncertain. Historically, there have been many postulated cells of origin. This poorly circumscribed tumor likely originates from a pluripotent dermal mesenchymal cell with fibroblastic differentiation. Why DFSP occurs only in the skin and not in other organs in which fibrocytes are present is an unanswered question.