Rare syndromic presentations of PBMAH can be observed as part of dominantly inherited diseases such as multiple endocrine neoplasia type 1 (MEN1) [2–4], familial adenomatous polyposis (FAP) [5–7], hereditary leiomyomatosis and renal cell cancer (HLRCC) [8, 9], due to germline pathogenic variants of MEN1 (Menin), APC (Adenomatous Polyposis Coli) and FH (Fumarate Hydratase) genes, respectively, and others [10]. Here, FH is linked to hereditary leiomyomatosis and renal cell cancer.