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Recent studies show that the prevalence of germline pathogenic and likely pathogenic variants (also known as mutations) in DNA repair genes in metastatic prostate cancer is higher than previously recognized and higher than in unaffected men. Specific gene dysfunction is important in prostate cancer initiation and/or evolution to metastases. This article reviews key literature on individual genes, recognizing BRCA2 as the gene most commonly altered in the metastatic setting. This article discusses the importance of representative and diverse inclusion, and efforts to advance management for at-risk carrier populations to maximize clinical benefit., Competing Interests: Disclosure A.O. Sokolova receives research funding from NIH/NCIT32CA009515. E.I. Obeid receives research funding from the American Cancer Society, Eileen Jacoby Foundation, Genetech, Merck, NCCN Foundation Young Investigator Award, and Newtown Foundation; and has a consultancy or advisory role to Daiichi Sankyo, Ethos Immunomedics, Foundation Medicine, Incyte, Novartis, OncLive, Pfizer, and Puma. H.H. Cheng receives research funding from the PNW SPORE CA097186, Prostate Cancer Foundation, NIH/NCI P30 CA015704, DOD W81XWH-17-2-0043; receives research funding to institution from Clovis Oncology, Club Foundation, Janssen, Medivation and Sanofi; has a consultancy role to AstraZeneca; and receives royalties from UpToDate. The authors have nothing else to disclose, (Copyright © 2021 Elsevier Inc. All rights reserved.)