Over 900,000 new cases of prostate cancer (PCa) are diagnosed worldwide every year. PCa is rarely diagnosed in men younger than 50 years, but its incidence rises rapidly thereafter. Excluding advanced age, the strongest risk factor for the disease is a family history of PCa suggesting the importance of genetic factors in disease development. Genome wide association studies (GWAS) have identified over 70 susceptibility loci associated with modest relative risks of PCa, which taken together, explain about 30% of the familial PCa risk. Rarer genetic variants conferring higher PCa risks have also been identified, such as BRCA1, BRCA2, HOXB13, and the Lynch Syndrome genes. Although the majority of PCa patients may only need active surveillance or are cured with radical primary treatment, others will eventually succumb to advanced disease. In fact, PCa accounts for the second commonest cause of male cancer-related deaths in the United States and the sixth worldwide, with more than 250,000 deaths a year. Thus, it is essential to identify upfront those patients with a lethal form of PCa and several studies have suggested that BRCA1 and BRCA2 mutation carriers may constitute one such population.