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Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare kidney tumor that tends to occur in middle-aged and older women and is characterized by a distinctive histological appearance. Most of them were incidentally detected. A 26-year-old female patient was referred to in our clinic due to intermittent left lower back pain for 2 months and left renal mass. Abdominal computed tomography showed a cystic enhanced heterogenic left renal mass about 5 cm in the largest diameter was extending from the renal pelvis to the ureter and causing gross hydronephrosis of the left kidney. The mass treated with transperitoneal laparoscopic nephroureterectomy and bladder cuff resection. Histopathological evaluation revealed MESTK. In our patient, MESTK successfully and without any complication be treated by minimally invasive surgery. We believe that the fact that the tumor can mimic the urothelial-cell carcinoma of the kidney in radiological appearance, as seen in our case, should be taken into consideration.