Cutaneous carcinosarcomas (CCSs) are a heterogeneous group of tumors with distinct epithelial and mesenchymal malignant componets. Although well characterized in other anatomic location, carcinosarcomas of the skin are rare; the histogenesis and biological aspects of CCSs remain poorly understood. The diagnosis can be challenging. Due to the limited number of reported cases, there is no consensus regarding prognostic or treatment. We present a novel case of primary CCS and a brief review of the relevant literature. The patient is a female of 61 years, without significant medical history, showing an asymptomatic tumor formation in the scalp region, clinically diagnosed as trichilemmal cyst. Histopathological examination revealed a biphasic tumor proliferation, constisting of two components, both malignant – an epithelial and a mesenchymal one; we performed immunohistochemical tests and we made the diagnosis of primary CCS. The immunohistochemical studies were of crucial importance for the diagnosis.