Abstract: Hereditary colorectal cancer is a disease caused by pathogenic germline variants in susceptibility genes. Lynch syndrome, previously called hereditary nonpolyposis colorectal cancer (HNPCC), is the most common type of hereditary colorectal cancer. In recent years, genome analysis provided an insight into the genetic basis of the disease. Here, we review the most recent advances in diagnosis and treatment of Lynch syndrome, including screening strategies, redefinition of Lynch-like syndrome, immunotherapy, and chemical prevention of Lynch syndrome.