CT and MRI have become the most common and most important tools for the diagnosis of renal AML, because of the low specificity of the symptoms and the limited value of the clinical presentation. If a kidney mass contains fat, renal AML is the first indication, as the incidence of fat in renal cell carcinoma (RCC) is very low. Ultrasound is not generally recommended for the diagnosis of renal AML, but can be used as a follow-up test if the diagnosis of renal AML is clear on CT. Renal AML is a relatively rare benign tumor that can be disseminated or combined with tuberous sclerosis (TSC) or lymphangioleiomyomatosis (LAM). The presence of a fatty component in the mass on CT or MRI is an important marker for the diagnosis of renal AML, but some renal AML with low fat content is often not easily detected on CT and plain MRI, so MRI chemical shift isotropic and antiphase imaging is needed to clarify. For asymptomatic renal AML with tumor size <4 cm, active follow-up is the first choice, while only those with clinical symptoms such as hematuria, large tumor size and easy rupture and bleeding should be considered for interventional treatment. Surgical resection and arterial embolization are currently the most used treatments, but all should be decided on a case-by-case basis for each patient. In patients with renal AML combined with TSC and LAM with poor residual renal function, oral mTOR inhibitors have been shown to be effective in slowing disease progression, and patients tolerate the side effects reasonably well. Of course, there are still many unknowns regarding the treatment of renal AML, and for the time being, the combination of TSC or LAM should be determined first, and then the treatment plan should be tailored to the patient's condition.