Hereditary melanoma is most often related to familial atypical mole melanoma syndrome (FAMMM), which tends to occur in multiple members of an equivalent family (also referred to as familial melanoma). Mutations during a number of genes involved in cell proliferation and melanin biosynthesis increase the danger of melanoma development. Inheritance of those genes may manifest as multiple relations with melanoma, as multiple primary melanomas during a given individual; or as a primary melanoma with onset at an early age. In general, the risk of melanomas in people who have one or more first-degree relatives with melanoma is approximately 5–12%. Other hereditary cancer syndromes, like hereditary breast and ovarian cancer syndrome (HBOC), Li–Fraumeni syndrome (LFS), etc., can also increase the danger of melanoma. Most of the gene mutations are transmitted in an autosomal dominant fashion. The identification of people at risk of developing hereditary melanoma is vital so as to implement strategies for reducing the burden of early disease.