A remarkable clinical response to immune checkpoint inhibitors of MSI-H high grade Mucoepidermoid carcinoma of the parotid gland with multiple metastases in the abdominal cavity

• We first to report a 64-year-old woman of Mucoepidermoid carcinoma of MSI-H high grade Mucoepidermoid carcinoma of the parotid gland with multiple metastases in the abdominal cavity treated with Capecitabine plus sintilimab and response evaluation was partial response (PR). • For MSI-H high grade Mucoepidermoid carcinoma of the parotid gland, anti-PD-1 immunotherapy could be considered to further improve therapeutic qualities and prognosis. • Our case showed an encouraging efficacy of Capecitabine plus sintilimab combination therapy. This promising avenue deserves further study to apply this combination regimen to clinical practice.