African Americans (AAs) have the highest incidence and mortality of colorectal cancer (CRC) in the United States (US). Few data are available on genetic and non-genetic risk factors for CRC among AAs. Little is known about cancer risks and mutations in mismatch repair (MMR) genes in AAs with the most common inherited CRC syndrome, Lynch syndrome. We aimed to characterize phenotype, mutation spectrum, and risk of CRC in AAs with Lynch Syndrome.