Rare loss of function (LoF) germline mutations in BRCA2 have convincingly been implicated as contributing to both FH of PCa and increased likelihood of aggressive disease with poor prognosis, whilst lower mutational frequencies or less consistent evidence has also been presented for a small subset of additional DNA repair genes including ATM, BRCA1, BRIP1, CHEK2, GEN1, MSH2, NBN, PALB2 and RAD51D[5], [6], [7]. The gene discussed is GEN1; the disease is posterior cortical atrophy.