Epithelial-myoepithelial carcinoma was first described by Danath et al. in 1972 and is classified as a rare low-grade biphasic neoplasm of the salivary glands. This case report presents a male patient who had a lesion in the oral mucosa with a history of recurrence of the tumor. The outcome resulted in a profile consistent with an epithelial-myoepithelial carcinoma with a high degree of transformation. The case highlights the importance of histopathological evaluation of oral lesions, which occasionally may not present typical clinical aspects of malignant lesion.