Epithelial-myoepithelial carcinoma (EMC) is a rare type of malignant tumor, accounting for about 1% of all salivary gland tumors arising most commonly in the parotid gland, but it has also been described in the submandibular gland as well as in minor salivary glands and palate. A biphasic cell arrangement formed by an inner layer of duct-forming epithelial cells and an outer layer of myoepithelial cells is the histological hallmark

Herein, we describe the case of a 44 year old patient diagnosed with an epithelial-myoepithelial carcinoma of the right parotid treated surgically with positive margins, followed by adjuvant radiotherapy; a next generation sequencing showed a HRAS Q61R mutation; three years and a half later he relapsed with lung metastasis treated by surgical excisions along with a platinum based therapy coupled with 5-FU but two years later new lung lesions were seen and the platinum based therapy coupled with 5-FU was adopted again with a stable disease; hence he was started on oral cyclophosphamide for one year; once again, those lung lesions kept on increasing in size and number and once again the platinum based therapy coupled with 5-FU was prescribed but stopped after 6 months for intolerance and major asthenia, and a we decided to stop the treatment . After a median follow up of three years, the disease is always stable and there’s no evidence of loco-regional recurrence.