Tuberous sclerosis syndrome (TSC) is an autosomal dominant tumor suppressor gene syndrome characterized by neurologic disease, benign tumors in multiple organs, including renal angiomyolipomas, and pulmonary lymphangioleiomyomatosis (LAM), which is due to inactivating mutations in either TSC1 or TSC2[1]. Here, TSC2 is linked to kidney angiomyolipoma.