Within the melanoma-dominant syndromes caused by cyclin-dependent kinase inhibitor 2A (CDKN2A), and BRCA1-associated protein 1 (BAP1), pathogenic mutations in these genes cause increased incidence of other cancer types (e.g., pancreatic cancer, neurological tumors, renal cell carcinoma, mesothelioma) but with lower penetrance than melanoma [2]. The gene discussed is CDKN2A; the disease is hereditary clear cell renal cell carcinoma.