We report a patient with ipsilateral synchronous renal cell carcinoma and urothelial carcinoma of the left renal pelvis and ureter. The patient underwent nephrectomy 9 years earlier due to renal cell cancer of the right kidney. Only 25 cases of ipsilateral synchronous renal cell carcinoma and urothelial carcinoma have been reported in the literature. The median age at diagnosis was 65 years, hematuria was the most common presentation and 24% of patients had metastases at first examination. Approximately one fourth of the patients had a history of cigarette smoking. There is no proof about higher histopathological grade of malignancy or specific histological pattern of these tumors when they occur synchronously. In the literature there is no report on the synchronous occurrence of renal cell carcinoma and urothelial carcinoma of both the renal pelvis and ipsilateral ureter.