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Women with a family history of breast cancer are at significantly increased risk of psychological distress, and there are potential benefits to routine psychological screening in women attending familial cancer clinics. Standard psychological screening measures may have limited sensitivity and specificity. Sensitivity and specificity of screening may be improved if screening includes additional routine assessment of empirically demonstrated risk factors for psychological distress specific to this population. To this end, we undertook a systematic literature review to identify demonstrated risk factors for psychological distress amongst high-risk women. Published studies of unaffected women with a family history of breast cancer were included if they: assessed sociodemographic, family history or psychological correlates of psychological distress; and included psychological and emotional outcomes as primary outcome measures. A total of 16 studies met the inclusion criteria. Several factors are empirically demonstrated risk factors in this population: (i) overestimating one's risk of developing breast cancer by more than 50%; (ii) mother's diagnosis with breast cancer; (iii) death of parent from breast cancer; (iv) having acted as caregiver for mother with breast cancer; and (v) having experienced a breast-cancer-related life event in the past year. A screening tool that incorporates these risk factors is presented and its potential role in familial cancer clinics is discussed.