Background: Metastasis of renal cell carcinoma (RCC) to the oral cavity is rare. Given the poor prognosis of metastatic RCC, treatment choice is difficult. Here, we report a case of RCC metastasis to the maxillary bone, and provide a detailed literature review regarding the patient characteristics, treatments and outcomes of RCC metastasis to the oral cavity.Case presentation: An 89-year-old Japanese man presented with an 8 × 8-mm granulomatous tumor with palpable pulsation in the left upper gingiva, which had been clinically suspected as an arteriovenous malformation. The patient had undergone left nephrectomy for clear cell carcinoma 7 years prior. Pulmonary metastasis had appeared 3 years later. The patient underwent tumor resection of the maxilla after intravascular embolization, and the tumor was histopathologically diagnosed as a metastasis of clear cell RCC to the maxillary bone. Seventeen months after surgery, he died because of pulmonary metastasis without evidence of recurrence in the oral cavity.Conclusion: Our literature review reveals that oral metastatic lesions of renal cancer often exhibit rapid enlargement and cause severe symptoms, such as dysphagia and bleeding. Although oral metastasis of RCC has a poor prognosis due to the presence of concurrent disseminated metastases, surgical therapy may be recommended because of its high local control rate and ability to maintain quality of life.