Tuberous Sclerosis Complex (TSC) is an autosomal dominant disease caused by germline loss-of-function mutations in the TSC1 or TSC2 genes, leading to neurologic disease (seizures, autism, and cognitive disability) and lesions in multiple organs including the brain, skin, heart, lungs (lymphangioleiomyomatosis), and kidneys1,2. Here, TSC2 is linked to lymphangioleiomyomatosis.