Mucoepidermoid carcinoma (MEC) represents 15% of all salivary glands malignancies. Metastatic disease at diagnosis is observed in less than 5% of the cases. The lung is the most commonly involved site. This is the first reported case of high-grade MEC of the salivary gland with skin metastases at diagnosis. This feature was associated with a chemoresistant and aggressive behaviour. Differential diagnosis between metastatic MEC and primary skin MEC is essential for therapeutic management and prognosis.