Literature regarding screening behaviour in individuals with a family history of colorectal cancer was reviewed, in order to determine the prevalence of screening in this population and identify factors associated with screening participation. Four electronic databases were searched from 1994. Thirty papers met the inclusion criteria, including 3 community surveys, 13 studies on first-degree relatives of colorectal cancer patients, and 14 studies on genetic services for colorectal cancer risk assessment. Individuals with a family history of colorectal cancer, who have not received risk assessment, frequently have never had any form of screening for colorectal cancer. Uptake of endoscopic screening when offered to individuals identified as being at increased risk was generally high (often >60% participation). Having a medical recommendation to screen, a stronger family history and perceiving fewer barriers to screening were identified as predictors of screening behaviour. Existing data suggest that use of screening tests in individuals with a family history of colorectal cancer is variable, and our understanding of factors associated with screening behaviour is limited. A number of methodological problems in research to date were identified, and further research is needed in order to inform interventions to support sustained screening participation in this population.