The aim of this guideline is to provide a potential management strategy of women at high risk for breast cancer development. A summary of the available evidence is presented, including genetic risk assessment, chemoprevention, risk-reducing surgery and radiological screening, based on risk assessment of the individual. Recent progress in the diagnostic methods and therapeutic options for breast cancer does not prevent the death of at least one third of these patients from their disease. The focus on breast cancer prevention, especially for the group of women that is designated as high-risk, may reduce mortality. The determination of the group of women who are more likely to develop breast cancer will allow a targeted specific counselling and the application of preventative measures. All Cancer Centres and Units should develop an integrated network of breast cancer care using common clinical guidelines, management protocols and strategies of care (Recommendation grade D). All Breast Units should have a protocol for the management of women at high risk (Recommendation grade D).