Hereditary nonpolyposis colorectal cancer (HNPCC), or Lynch syndrome, is an autosomal dominant syndrome characterized by onset of colorectal cancer (CRC) at an early age, right-sided predominance, excess synchronous and metachronous colorectal neoplasms, and extracolonic neoplasms. It is the most common of the hereditary CRCs, so the practicing surgeon should expect to encounter patients with this disease. The diagnosis of HNPCC, which begins with a complete family history and a high index of suspicion by the clinician, has important implications in the management and surveillance of not only the affected individual but also for the individual's family. In this article, the diagnosis and management of Lynch syndrome will be reviewed, with emphasis on the implications for the surgeon.