ABSTRACT Purpose Renal cell carcinoma (RCC) is a malignant tumor that metastasizes early, and patients often present with metastatic disease at the time of diagnosis. The aim of our evaluation was to assess the diagnostic and differential diagnostic relevance of metastatic renal cell carcinoma (RCC) with particular emphasis on head and neck manifestations in a large patient series. Patients and methods We retrospectively evaluated 671 consecutive patients with RCC who were treated in our urology practice between 2000 and 2013. Results Twenty-four months after diagnosis, 200/671 (30%) of RCC had metastasized. Distant metastases were found in 172 cases, with 22 metastases (3.3%) in the head and neck. Cervical and cranial metastases were located in the lymph nodes (n=13) and in the parotid and the thyroid gland, tongue, the forehead skin, skull, and paranasal sinuses (n=9). All head and neck metastases were treated by surgical excision, with 14 patients receiving adjuvant radiotherapy and 9 patients receiving chemotherapy or targeted therapy at some point during the course of the disease. Five patients (23%) survived. The mean time of survival from diagnosis of a head and neck metastasis was 38 months, the shortest period of observation being 12 months and the longest 83 months. Discussion and conclusion Our findings show that while RCC metastases are rarely found in the neck, their proportion among distantly metastasized RCC amounts to 13%. Therefore, the neck should be included in staging investigations for RCC with distant metastases, and surgical management of neck disease considered in case of resectable metastatic disease. Similarly, in patients presenting with a neck mass with no corresponding tumor of the head and neck, a primary tumor below the clavicle should be considered and the appropriate staging investigations initiated.