Renal cell carcinoma (RCC) is often diagnosed in advanced stages and a third of patients have distant metastasis at diagnosis. Metastasis may be the first evidence of clear cell RCC in many cases. RCC most often metastasises to the lung, liver, bone, brain and thyroid; however, metastatic disease to the oral cavity, especially the mandible, is rare. The purpose of this study is to report a case of clear cell RCC metastatic to the mandible and review the literature. The mandible lesion underwent radical excision in this case. Notably, no metastatic lesions were detected in the lungs and liver in this patient until 15 months after the mandibulectomy. The patient lived for around 2.5 years after the diagnosis of RCC.