Adrenocortical carcinomas (ACC) are mainly sporadic, but may be found in 3% to 5% of some genetic syndromes such as Li-Fraumeni syndrome (LFS), Beckwith-Wiedemann syndrome (BWS), Lynch syndrome, familial adenomatous polyposis (FAP), and in <1% of multiple endocrine neoplasia type 1 (MEN1) syndromes.[1,2] Germline mutations in BRCA1 (MIM 113705) and BRCA2 (MIM 600185) genes account for cancer predisposition in majority of families with breast only or breast–ovarian cancer families.[3] To date, BRCA2 mutation in ACC has never been reported. This evidence concerns the gene BRCA2 and Beckwith-Wiedemann syndrome.