Our updated systematic review and meta-analysis investigates the frequency of germline and somatic BRCA1 and BRCA2 mutations in patients with prostate cancer (PC), with subgroup analysis according to the type of mutation (germline or somatic mutations; mutation of BRCA1 and/or BRCA2) and according to the disease setting (any stage PC or metastatic PC or metastatic castration-resistant PC). The gene discussed is BRCA1; the disease is pachyonychia congenita.