Epithelial-myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined. Epithelial-myoepithelial carcinoma (EMCa) is a low-grade malignant tumour. According to literature, most commonly occurs in salivary glands, particularly in parotic gland, but it can also occur in unusual locations such as breast, lachrymal gland, nose, paranasal sinus, lung, bronchus and, as in our case, trachea. There are no many documented case reports of a primary myoepithelial carcinoma in the trachea. We report a case of a 34-year-old man diagnosed with this unusual location of an epithelial-myoepithelial tumor. The tumour was removed by segmental tracheal resection and end-to-end anastomosis.