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Most ACCs are sporadic, but a small percentage of cases are due to hereditary cancer syndromes such as Li\u2010Fraumeni syndrome (LFS), Lynch syndrome (LS), and familial adenomatous polyposis (FAP). Multiple endocrine neoplasia type 2A (MEN2A) is an inherited disorder that predisposes to medullary thyroid cancer, pheochromocytoma, and parathyroid hyperplasia. We present here a case of ACC with both LS and MEN2A; the family and medical history were consistent with Lynch. This is, to our knowledge, the first report of a patient with ACC associated with germline mutations in <jats:italic>RET<\/jats:italic> and <jats:italic>MSH2<\/jats:italic>, and no phenotypical characteristics of MEN2A.<\/jats:p>","DOI":"10.1002\/ajmg.a.62099","type":"journal-article","created":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T06:22:07Z","timestamp":1613974927000},"page":"1282-1287","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Case report of adrenocortical carcinoma associated with double germline mutations in <scp><i>MSH2<\/i><\/scp> and <scp><i>RET<\/i><\/scp>"],"prefix":"10.1002","volume":"185","author":[{"given":"Margarita","family":"Raygada","sequence":"first","affiliation":[{"name":"Pediatric Oncology Branch, Center for Cancer Research National Cancer Institute, Clinical Center, National Institutes of Health  Bethesda Maryland, DC 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