Risk factors for atypical endometrial hyperplasia include obesity, chronic anovulation diseases such as PCOS, and certain genetic factors such as pathogenic variants in DNA mismatch repair genes (MLH1, MSH2, MSH6, PMS2, and EPCAM) leading to Lynch Syndrome, Cowden Syndrome (PTEN pathogenic variant), and Peutz-Jeghers (STK11 pathogenic variant) [52]. The gene discussed is PMS2; the disease is Cowden disease.