In LAM, the absence of the TSC1–TSC2 complex leads to uncontrolled activation of mTOR.[5] Abnormal proliferation of smooth muscle-like cells (LAM cells) results, leading to development of tumors (mostly hamartomas) in various sites.[6,7] Although these mainly form in the lungs, extrapulmonary involvement also occurs, typically in the kidneys, retroperitoneum, and pelvic regions.[8,9] Cysts form in the lungs causing destruction of pulmonary architecture, whereas solid tumors develop in kidneys (angiomyolipomas) and retroperitoneal and abdomino-pelvic sites (lymphangioleiomyomas). Here, MTOR is linked to lymphangioleiomyomatosis.