Lynch syndrome (LS) is an autosomal dominant disease caused by pathogenic mutation of mismatch repair (MMR) genes that confers a predisposition to cancer development, and microsatellite instability (MSI) is its main feature in clinical assay. LS is responsible for most hereditary colorectal cancer. With the continuous improvement of molecular diagnostic technology, the accurate diagnosis and treatment of LS-related colorectal cancer through LS molecular detection has gradually become the focus of clinical attention. Despite the favorable prognosis of LS-related colorectal cancer, it is still an important task to further improve the screening and follow-up strategies for patients with family history of LS in our country. In addition, using the immunological characteristics of LS to guide its treatment and prevention is a new challenge for many scholars. Herein, the authors review the recent advances concerning LS-related colorectal cancer in the aspects such as the epidemiology, clinicopathologic features, screening diagnosis and treatment as well as prevention.