Metastases to the ovary occur in 0.8-9.7 % of colorectal cancer (CRC) cases (Hanna and Cohen in Clin Colorectal Cancer 3(4):215-222, 2004). The need to combine surgical resection of the primary tumor and bilateral oophorectomy is a matter of debate (Erroi et al. in J Surg Oncol 96(2):113-117, 2007). In a consecutive multi-hospital cohort of 30 CRC metastases to the ovary we came across four female patients (13 %; 95 % CI 3.6-34.1) with familial adenomatous polyposis (FAP). This number is high since the estimated incidence of FAP CRC is far below 1 % of all CRC and the expected incidence of FAP CRC that metastasized to the ovaries would thus be almost zero. In a second screen in nationwide databases we found that ovarian metastases occurred in at least 15 % of female FAP CRC cases. We provide now first evidence that especially in female FAP CRC patients bilateral oophorectomy during surgery should be discussed.