The association of renal angiomyolipoma and renal oncocytoma in the same kidney is rare, only 7 cases having been previously reported. We report a 69-year-old woman affected by the simultaneous presence of renal angiomyolipoma and oncocytoma in the right side with a decrease of renal function on the left side due to a stenosis of the superior polar branch that was successfully treated preoperatively by angioplasty. An enucleation of renal masses on the right side was performed with renal function preservation after 2 years.