This study provides an assessment of long-term breast and ovarian cancer screening behaviors and the variables associated with adherence with screening among women with or at 50% for having a BRCA1/2 mutation. Participants in the study included 112 women (33 mutation carriers and 79 at 50% risk). Data was collected through a mailed questionnaire, which included items to assess screening behaviors in the last 2 years, risk perception, cancer specific distress, adherence determinants, specific barriers, and cancer history. Statistical analysis included descriptive statistics and non-parametric tests to describe bivariate associations and regression analysis. Adherence rates were 72% for annual mammography, 21% for semi-annual clinical breast exam (CBE), 29% for monthly breast self-exam (BSE), and 19% for annual transvaginal ultrasound (US). Only one participant was adherent with semi-annual CA125. Variables that had a significant association (P < 0.05) with at least one screening modality included: a lack of time, marital status, education, cancer history, provider concern, perceived screening utility, confidence in ability to overcome barriers, cancer specific distress, and risk perception. Primary physician behavior, either in terms of screening recommendations or screening performance in the case of CBE, had significant independent association with adherence to mammography, CBE, and US screening recommendations. The results of this study highlight the essential role that primary physicians play in supporting their very high-risk patients' adherence. © 2003 Wiley-Liss, Inc.