A subject of endometrial cancer (case 3) with a germline pathogenic variant (MSH6 c.1126G>T) showed a loss of MSH6 expression, but retained microsatellite stability, suggestive of an MSH6‐specific phenomenon.33 Case 17, with a likely benign variant (MSH6 c.532C>T), revealed preserved tumor MSH6 and MSH2 expression, but showed MSI‐H and a loss of tumor expression of MLH1 and PMS2. Here, MSH6 is linked to endometrial cancer.