Based on their prevalence in previous studies [27–36] and our clinical experience in dermatology, we focus on Li-Fraumeni syndrome (LFS), Lynch syndrome (LS), hereditary breast and ovarian cancer (HBOC) syndrome, ATM-associated hereditary cancer syndrome, CHEK2-associated hereditary cancer syndrome, cancer predisposition associated with BRIP1 mutations, and hereditary leiomyomatosis and renal cell carcinoma (HLRCC) in this narrative review. This evidence concerns the gene ATM and hereditary leiomyomatosis and renal cell cancer.