The genetic predisposition to epithelial ovarian cancer can be distinguished in two different forms: familial breast and/or ovarian cancer; familial colon, endometrium, ovarian cancer or HNPCC syndrome (hereditary non polyposis colorectal cancer). The BRCA1 and BRCA2 genes are involved in familial breast and (or) ovarian cancer. Mutations of these two genes could explain 5.5% (2-7%) of ovarian cancers. The hMLH1, hMSH2, and hMSH6 genes are involved in the HNPCC syndrome. The mutations of these genes could explain 1% to 2% of ovarian cancers. The clinical management of women at ovarian cancer risk is variable and dependent on the predisposition going from regular examination until prophylactic oophorectomy in the presence of BRCA mutations.