Development of uroepithelial tumors after cyclophosphamide and azathioprine therapy in Wegener's granulomatosis (WG) have been reported in the literature but renal cell carcinoma (RCC), rarely. RCC associated with WG has been previously reported in a few cases. Most of them have simultaneous diseases. Here, we report a case, which developed RCC 8 years after initiation of WG. Long-term immunosuppressive treatment is a risk factor for the development of malignancies; it should be suggested that RCC in our patient might be due to immunosuppressive therapy.