There is evidence to suggest that increased levels of homocysteine play a significant role in vascular disease. It has been suggested that lowering homocysteine levels by dietary folate supplementation may reduce the risk of stroke and coronary heart disease. It is plausible that homocysteine may also play a role in the pathogenesis of abdominal aortic aneurysms (AAA) and that patients with this disease may benefit from folate supplementation. Our objective was to review the published work with regard to the role of homocysteine in the pathogenesis of AAA. Searches were carried out in published work in English with the keywords 'abdominal aortic aneurysm' and 'homocysteine'. There is evidence from in vitro and animal model studies that activation of metalloproteinases by homocysteine can influence aortic wall structure. Several case-control studies report an association between increased levels of homocysteine and the presence of an AAA. There are conflicting genotypic data concerning the association between methylenetetrahydrofolate reductase gene variants and AAA. Although there is evidence for an association between homocysteine and AAA, it is not strong enough to conclude that it plays a causal role in the pathogenesis of AAA. Further research is needed, given the potential benefit that simple vitamin supplementation may have for patients with AAA.