Melanoma is a highly aggressive malignant skin tumor that originates from melanocytes. Interactions between genetic,epigenetic and environmental factors have an important role in the etiopathogenesis of melanoma. In clinical practice, sporadic melanomas predominate, whereas hereditary ones account for approximately 10%. Most inherited melanomas develop due to mutations in the CDKN2A gene, which encodes for two tumor suppressor proteins involved in cell cycle regulation. This gene, along with CDK4, TERT, and POT1, are high-risk genes for melanoma. Among the genes that carry a moderate risk are MC1R and MITF, whose protein products are involved in melanin synthesis. Since, in addition to genetic factors, the influence of the environment also contributes to the development of melanoma, it is necessary to include risk groups in the process of genetic counseling. During counseling, in addition to the possibility of genetic testing, the importance of protecting the skin from UV rays, avoiding sun exposure and undergoing preventive examinations are discussed. Cancer screening exams cannot prevent the development of the disease, but allow its diagnosis at the earliest stage, when the survival rate is highest.