Research into the psychological effects of reproductive decision-making when an individual has a diagnosis of Marfan syndrome, a genetically inherited and potentially life-threatening condition, is rare and mainly quantitative in nature. In response, this study has investigated the experience of couples’ decisionmaking to have a baby when a woman has been diagnosed with Marfan syndrome. The study was conducted using unstructured interview data, analysed using the qualitative methodology of Interpretative Phenomenological Analysis (IPA). The participants were six couples with a female diagnosis of Marfan syndrome. Five couples with children provided retrospective data and one couple making the decision to have a baby provided live data. Four superordinate themes emerged: her decision is already made; creating an informed decision; finding psychosocial support; existing with the fallout of the decision. The experience of decision-making was complex and multidimensional for the males, females and couples. The women demonstrated a strong drive to have a baby despite the 50/50 odds of having a child with Marfan syndrome, whereas the men described the tension between wanting a child and coping with the potentially life-threatening risks for a woman in pregnancy and childbirth. A compelling account of the couples’ difficulties in exploring their options and assessing the risks with medical professionals emerged. It is argued that this research provides important insights for counselling psychologists and other professionals when working with individuals and couples affected by Marfan syndrome and other genetically inherited conditions at the critical time of coping with reproductive decision-making