Previous studies have shown that inactivating germline mutations of tumor suppressor genes including MEN1, APC and FH could result in PBMAH accompanied by other syndromic presentations such as primary hyperparathyroidism, neuroendocrine tumors, pituitary adenomas (MEN1 syndrome, multiple endocrine neoplasia type 1), colon polyps (FAP, familial adenomatous polyposis), leiomyomas, leiomyosarcomas and renal cancer (HLRCC, hereditary leiomyomatosis and renal cell cancer) [3]. The gene discussed is APC; the disease is Familial adenomatous polyposis.