ACC is rare with an incidence of 0.7 to 2.0 cases per million populations per year.[4] Analyses of inherited syndromes related to ACC led to the progress in the pathogenesis of ACC including the LFS due to germline TP53 mutations, the BWS due to the deregulation of imprinted genes in the chromosome 11p15.5 region, which contains the insulin-like growth factor 2 and the closed linked H19 gene in the imprinting center 1, the Lynch syndrome due to a defect in the mismatch repair system (MSH2, MSH6, MLH1), and more rarely the MEN1 (menin) and FAP syndromes (APC).[1,2]. The gene discussed is MSH6; the disease is adrenal cortex carcinoma.