Background: Colorectal cancer is the third in frequency and the second in mortality in developed countries. In Brazil, it is among the six more common malignant neoplasias. About 20% of colorectal tumors have some hereditary component.
Aim: This study presents a review of genetic and clinic aspects, as well as diagnosis and prevention of the hereditary non-polipomatous colorectal cancer, that is the more frequent form of hereditary colorectal cancer. This approach is important because, currently there are possibilities of management, prevention and surveillance specific to individuals at-risk for hereditary non-polipomatous colorectal cancer that can lead to a great improvement in patients' survival and their at-risk relatives.