Although the majority of breast cancers are sporadic, it is estimated that between 5% and 10% of cases are hereditary and mostly associated with BRCA 1 and BRCA 2 mutations. Women with BRCA 1 or BRCA 2 mutation present up to 95% increased risk of breast cancer and are advised to take preventive measures. Surveillance, chemoprevention and prophylactic surgery (mastectomy and oophorectomy) are risk-reducing strategies. This literature review aims to respond to two major questions: 1) to what extent is the decision-making for prophylactic mastectomy in women with the BRCA mutation a more relevant option than other strategies, and 2) what are the major factors influencing the decision for mastectomy? For this purpose, 27 published articles were reviewed. Results show: a) the decision for mastectomy is more frequent in women carrying BRCA 1/2 mutation than in non-carriers; mastectomy being less frequent than oophorectomy; b) the decision-making is significantly associated with BRCA mutation, parity, age and family history; c) reasons underlying women decision-making relate to anxiety toward the risk of cancer, concerns with surgery, body-image and sexuality. It is discussed that, due to the complexity of the decision-making process, these cases require a careful and meticulous approach regarding information provided, and in addressing concerns.