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Male-to-female transgender persons (trans-women) receive livelong cross-sex hormonal treatment in order to induce and maintain secondary female characteristics. One of the concerns of long-term estrogen treatment is the induction of carcinomas of estrogen-sensitive tissues such as the breast. BRCA1 mutations have been shown to account for a large proportion of inherited predispositions to breast cancer.The aim of this case report is to discuss the hormonal and surgical options in the treatment of trans-women with a genetic predisposition for breast cancer.We describe a case of a trans-woman who was found to be a carrier of a BRCA1 mutation.The patient underwent a breast augmentation. She refused a prophylactic mastectomy followed by a primary breast reconstruction. She also underwent a vaginoplasty and a bilateral castration. Androgen blocking treatment was stopped after surgery; estradiol treatment however was continued.This case points to the importance of routine investigation of family history in trans-women. Trans-women with BRCA mutations should be carefully monitored and if cancers develop, this should be reported. Follow-up should be according to the guidelines for breast cancer screening in biological women, and the guidelines for prostate cancer and colon cancer screening in men. Colebunders B, T'Sjoen G, Weyers S, and Monstrey S. Hormonal and surgical treatment in trans-women withBRCA1 mutations: A controversial topic. J Sex Med 2014;11:2496–2499.