It is due to pathogenic DNA alterations in either TSC1 (OMIM 605284) or TSC2 (OMIM 191092) genes and is characterized by benign tumors (hamartomas) in distinct organs, mainly the brain, skin, heart, kidneys, and lungs.1, 2 The TSC1 gene (9q34) encodes hamartin, and TSC2 (16p13.3) codes for tuberin. This evidence concerns the gene TSC2 and benign neoplasm.