The lack of hamartin or tuberin leads to the constitutive activation of mTORC1 in TSC-associated lesions, which include cutaneous lesions, renal angiomyolipoma (AML), intraventricular subependymal giant cell astrocytoma, cardiac rhabdomyomas, and pulmonary lesions, whose main manifestation in women is lymphangioleyomiomatosis (LAM), a rare progressive cystic lung disease that can occur in a sporadic form or associated to TSC [3]. Here, TSC2 is linked to lymphangioleiomyomatosis.