The initial presentation of a cholangiocarcinoma (CC) as trismus due to metastasis to the parotid gland is extremely rare and no previous reports have been found in the literature. A 29-year-old woman presented trismus that initiated 2 months before admission, just after superior left third molar extraction. Physical examination revealed severe trismus and a mass in the parotid gland. Computed tomography (CT) showed a heterogeneous mass in the left parotid gland. The next day she presented jaundice, bilirubinuria, and fever, followed by massive bleeding, intravascular disseminated coagulation, and respiratory insufficiency. Two days later she died. At autopsy, the parotid tumor was identified as a metastasis from a CC of the extrabiliary tract. To our knowledge, this is the first reported case of a metastatic CC that initially presented as trismus due to a mass in the parotid gland. This case represents a rare but important diagnosis that otolaryngologists and oral surgeons should add to the catalog of uncommon causes of trismus.