Interestingly, new evidence of a significant association with cancer risk has been described [10] and recent studies reassessed the association of K3326X BRCA2 mutation with the risk of developing melanoma, urothelial, pancreatic, breast and ovarian cancers [11,12,13,14], while its role as modifier of genetic penetrance has been also reported [15]. This evidence concerns the gene BRCA2 and ovarian cancer.