Renal malformation tumor is a relatively common benign tumor of the kidney caused by an abnormal proliferation of the vascular smooth muscle of the kidney. Since it is a benign tumor, it does not require special treatment if it is relatively small in size. However, if the size reaches 4 centimeters or more and causes a series of symptoms such as pressure pain and bleeding, it needs to be treated promptly. Nephrectomy, kidney unit preservation surgery, kidney transplantation, radiofrequency ablation, interventional embolization, and hemodialysis are some of the more common treatments for malignant tumors. Nephrectomy is suitable for patients with tumor at the edge of kidney and tumor less than 4 cm, while nephrectomy is suitable for patients with larger tumor and to preserve kidney function and prevent lymph node invasion. Kidney transplantation or hemodialysis is suitable for patients with tumor rupture and bleeding or bilateral lesions leading to renal failure. As for which one to choose for treatment, clinicians need to develop the best treatment plan according to each individual’s specific situation. The prognosis after treatment of renal malformation tumor is mostly good, but regular review is required after treatment and continuous antibiotics are needed to prevent infection. After the treatment of renal malformation tumor, it is necessary to pay attention to rest and diet, not to do strenuous exercise, especially not to drink alcohol and stay up late, and to go to the hospital regularly for kidney ultrasound and kidney function review.