Increasingly, women with a positive family history of breast cancer are being targeted for cancer prevention and control efforts. Two findings have been demonstrated consistently across studies of this high risk population. First, these women often have high levels of psychological distress, as well as persistent and intrusive worries about developing breast cancer. Second, despite their increased risk for developing breast cancer, a substantial proportion of these women do not adhere to recommended breast cancer screening guidelines. There is growing evidence that psychological distress is an important barrier to adherence among high risk women. Despite this, little is known about how to intervene to improve psychological adjustment and adherence in this population. In the present paper, we review the literature on adherence and psychological adjustment in women who are at increased risk for breast cancer because of a positive family history of disease. This review provides the basis for a discussion of potential intervention strategies designed to increase adherence and reduce psychological distress in this population. Finally, we present some of the psychological implications of recent developments in genetic testing for breast cancer susceptibility.