Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, was approved as a pharmaceutical drug and has begun to be used in clinical practice. We reviewed the benefits and harms of mTOR inhibitors as a therapeutic drug for renal angiomyolipoma associated with sporadic lymphangioleiomyomatosis and summarized its clinical positioning. The body of evidence was evaluated and integrated by systematic reviews, and the recommendation was formulated, based on the methods described in the “Minds Manual for Guideline Development. Ver.2.0 (2016)” by the Japan Council for Quality Health Care. Becuase the use of mTOR inhibitors in adult women with renal AML associated with sporadic lymphangioleiomyomatosis is expected to reduce a tumor's size, they can be a therapeutic option when abdominal symptoms associated with an increase in tumor size are present, or when the risk of bleeding is considered high (weak recommendation based on low-quality evidence).