Carcinoma Ex-Pleomorphic Adenoma (CExPA) is a salivary gland carcinoma derived from a primary or recurrent benign pleomorphic adenoma (PA) extremely rare in minor salivary glands. In this paper, we report the case of a male afrodescendant patient, 37 years old, presenting a palatal irregular nodular lesion with approximately 3.5 cm diameter. The lesion had over two years of evolution, but started growing faster and presenting pain and ulceration in the last two months. The incisional biopsy revealed a typical pleomorphic adenoma with focal areas of nests of epidermoid and mucous cells, as well as microcyst formations, resembling the mucoepidermoid carcinoma (MEC). Immunohistochemical analysis revealed positivity for CK7, CK13, CK 14, p63 and Ki67 (about 30%), whereas α-SMA was restricted to the PA component. The diagnosis was CExPA (MEC-type). A discussion on the histopathological and immunohistochemical criteria for differential diagnosis of CExPA is provided in this work, hoping to contribute to a better knowledge and understanding of this rare malignant tumor.Key words:Salivary gland neoplasms, pleomorphic adenoma, adenocarcinoma, mucoepidermoid carcinoma, pathology, differential diagnosis.