A 41-year-old patient had a large and quick growing tumor of the scalp, known during many years. The histopathologic and immunohistochemical evaluations lead to the diagnosis of a cutaneous carcinosarcoma or sarcomatoid carcinoma. In spite of the surgical treatment, the evolution was rapidly fatal with widespread metastasis. From data of the literature, we discuss the diagnosis and histogenesis of this rare cutaneous tumor.