There are numerous genetic syndromes that result in an important predisposition to the occurrence of melanoma: Familial atypical multiple mole melanoma (FAMMM), Xeroderma pigmentosum (XP), BAP1 tumor predisposition syndrome (BAP1), Shelterin complex genes hereditary cancer syndrome (Shelterin), MC1R polymorphism (MC1R), and DICER1 (Table 1, Table 2 and Table S1). The gene discussed is DICER1; the disease is xeroderma pigmentosum.