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The occurrence of metastatic tumors in the orofacial region is rare and may represent the first clinical manifestation of occult malignant disease. An orofacial lesion diagnosed as a metastatic tumor from a renal cell carcinoma in a 68-year-old man is reported. This metastatic tumor caused significant facial asymmetry involving the parotid gland and mandible regions, and the patient died four months after diagnosis. Here, we discuss the clinical aspects, the diagnostic approach, and the importance of early diagnosis to obtain a better response to treatment and provide longer survival time.