Abstract
Renal cell carcinoma (RCC) is encountered in about 3% of all adult neoplasms. Presence of any kidney malformation can change the plan for surgical treatment of RCC with organ preserving surgery. We report a case of clear cell RCC in a horseshoe kidney. Computed tomography scan revealed a horseshoe kidney anomaly with a large mass in the left side. The diagnosis of RCC was confirmed by pathology and histology findings.