Lynch syndrome is the most common inherited colon cancer susceptibility syndrome. Lynch syndrome is characterized by a significantly increased risk for colon cancer and endometrial cancer and a smaller risk for several other associated cancers. Some periodic screening strategies, such as colonoscopy, reduce the incidence and mortality of Lynch syndrome. The aim of this review is to discuss the risks, surveillance tests and guidelines for the management of colonic and extracolonic tumors associated with Lynch syndrome. For extracolonic cancer, a benefit of surveillance is evident only for endometrial cancer. No definitive data show efficacy of chemopreventive drugs, although aspirin is a promising drug. In this review, the available evidence on the different screening strategies in Lynch syndrome will be discussed. Furthermore, the clinical and biological characteristics of this disease and their potential impact on prevention in individuals at risk are analyzed.