Colorectal cancer (CRC) is a major cause of morbidity and mortality in the world. Up to 30% of CRCs have evidence of a familial component and about 5% are thought to be due to well-characterized inherited mutations. This review will focus on recent developments in the understanding of the individual hereditary colorectal cancer syndromes including Lynch Syndrome, Familial Colorectal Cancer Type X, Familial Adenomatous Polyposis, MutYH associated polyposis, Peutz-Jeghers Syndrome, Juvenile Polyposis Syndrome, PTEN Hamartomatous Syndrome, and Serrated Polyposis Syndrome. Advances within the area of hereditary colon cancer syndromes paint a picture of a rapidly moving, rapidly maturing and increasingly collaborative field with many opportunities for ongoing research and development.