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Epithelial-myoepithelial carcinoma (EMC) is a rare tumor with predilection for the major salivary glands, mainly the parotid gland. It is a tumor of low grade malignancy, and despite its tendency to local recurrence with low metastatic potential, rare cases of EMC may have an aggressive behavior with distant metastasis. Treatment consists primarily of complete surgical resection whenever possible followed by radiotherapy (RT) in an attempt to prevent local recurrence. The authors describe two cases of patients with EMC from the parotid gland. Despite treatment and good local response both later developed pulmonary metastases, and also later cerebral metastization. In the sequence of these clinical cases, a literature review is made.