The reported prevalence was 1.44% in the healthy population and 2.1% in the cancer population, suggesting a role of germline monoallelic MUTYH variants in cancer development; a high frequency of monoallelic MUTYH variants was found in adrenocortical carcinoma (ACC), esophageal carcinoma, and sarcoma. The gene discussed is MUTYH; the disease is adrenal cortex carcinoma.