In total, 168 families fulfilling the definition of familial melanoma were tested for CDKN2A, CDK4, BAP1, and POT1. The median age at the earliest melanoma diagnosis in families was 40 years (range 12–78 years), 60% of families had ≥3 cases of melanoma, and 40% had multiple primaries. This evidence concerns the gene POT1 and melanoma.