Distant metastasis of renal cell carcinoma (RCC) to the parotid region is extremely rare, particularly as an initial presentation. In the present study, we report a rare case of parotid region metastasis from RCC as an initial presentation in a 44-year-old female who presented with a painless lump in the right parotid region. Investigation revealed RCC in the left renal region and metastasis to the right iliac area. A radical nephrectomy was performed but the patient refused any further treatment. After seven months, the patient reappeared with systemic multiple metastases, with the exception of previous metastases that were enlarging significantly. On admission, interleukin-2 and local radiotherapy were administered. However, oral mucositis occurred. Targeted therapy with sunitinib was recommended.