Syndromes such as Hereditary Breast and Ovarian cancer (HBOC) syndrome, BAP1 tumor predisposition syndrome, Lynch syndrome (LS), PTEN hamartoma tumor syndrome (PHTS), Bannayan–Riley–Ruvalcaba syndrome (BRRS), Cowden syndrome (CS), Proteus syndrome (PS), Gorlin syndrome (GS), Peutz–Jeghers (PJ) syndrome, and familial adenomatous polyposis (FAP) often exhibit distinctive dermatological features that can serve as early clinical indicators of an underlying genetic predisposition to malignancy. The gene discussed is BAP1; the disease is hereditary breast ovarian cancer syndrome.