Tuberous Sclerosis Complex (TSC; OMIM #191100, *605284, #613254, *191092) is a multisystemic disorder generally caused by germline variants in TSC1 or TSC2. Multiple renal and extra-renal angiomyolipomas (AMLs) represent one of the characteristic manifestations of TSC. Here, TSC1 is linked to tuberous sclerosis.