Men with BRCA1/2 mutations have an increased risk of prostate cancer (PCa)1 with an estimated relative risk of 1.8 to 4.5 fold for BRCA1 and 2.5 to 8.6 fold for BRCA2 mutation carriers.2, 3 Numbers of men with PCa attributed to BRCA1 or BRCA2 is relatively small but rising (approximately 2% of men diagnosed under the age of 55).1 This is clinically important as men with BRCA2 mutations present with aggressive PCa at a younger age and have poor survival.4 Awareness of PCa risk is increasing, becoming a standard part of genetic counselling for men at risk of inheriting a BRCA1/2 mutation. The gene discussed is BRCA1; the disease is posterior cortical atrophy.