Individuals at high risk for developing breast and/or ovarian cancer are faced with difficult decisions regarding genetic testing, cancer prevention and/or intensive surveillance. Large interindividual differences exist in the uptake of these health-related services. This paper is aimed at understanding and predicting how people emotionally and behaviourally react to information concerning genetic predisposition to breast/ovarian cancer. For this purpose, the self-regulation model of illness representations is elaborated. This model suggests that health-related behaviour is influenced by a person's cognitive and emotional representation of the health threat. These representations generate coping behaviour aimed at resolving the objective health problems (problem-focussed coping) and at reducing the emotional distress induced by the health threat (emotion-focussed coping). Based on theoretical considerations and empirical studies, four interrelated attributes of the cognitive illness representation of hereditary breast/ovarian cancer are described: causal beliefs concerning the disease, perceived severity, perceived susceptibility to the disease and perceived controllability. The paper also addresses the complex interactions between these cognitive attributes, emotional distress and preventive health behaviour.