Endometrial cancer is the 4th most common cancer in women in France, with an increasing incidence partly due to the increase in diabetes and obesity. A genetic predisposition is found in approximately 5% of cases, notably Lynch syndrome, Peutz-Jeghers syndrome, hamartomatous tumor syndrome (Cowden syndrome), and BRCA1/2 mutations. Although no organized screening is in place for endometrial cancer in the general population, individual screening is recommended for most of these at-risk populations. Prophylactic surgery is a key strategy to reduce the risk of endometrial cancer in high-risk women. For patients with Lynch syndrome, recommendations favor a total hysterectomy with bilateral salpingo-oophorectomy starting at age 40, after childbearing is complete. Hormonal supplementation with natural estrogens is important until the physiological age of menopause to reduce bone and cardiovascular risks and to improve the quality of life, including sexual health, for these women. In Cowden syndrome and Peutz-Jeghers syndrome, the lack of data limits surgical recommendations, although prophylactic hysterectomy may be considered starting at age 40, once childbearing is complete. For patients with BRCA1/2 mutations, while risk-reducing adnexal surgery is recommended, hysterectomy remains debated. It requires a personalized assessment with patients based on the benefit/risk balance.