Objectives The association between diagonal ear lobe crease (DELC) and cardiovascular disease was first suggested in 1973 although some studies have attributed this to confounding cardiovascular factors. This review looked to see if there is a significant association between DELC and angiography-confirmed coronary artery disease (CAD) independent of other risk factors. Design Systematic review and meta-analysis of selected studies using the PRISMA checklist. Setting 12 different hospitals with angiography in eight countries. Participants 4960 adult patients undergoing coronary angiography. Main Outcome Measures • Presence/absence of diagonal ear lobe crease • Diagnostic Odds Ratio • Sensitivity/Specificity Results 12 studies were included in the meta-analysis. Findings from our study suggest: • Patients with DELC have a 4x increased likelihood of having CAD (OR 4.61 P<0.00001). • The relationship between DELC and CAD was independent of age and all other conventional cardiovascular risk factors. • Bilateral DELC has a stronger association with CAD than unilateral DELC. • Presence of DELC has insufficient sensitivity / specificity to be used as a diagnostic test for cardiovascular disease but instead should be used as a risk marker. Conclusions We found that DELC is associated with CAD independently of other known cardiovascular risk factors including age. Histology studies indicate that atherosclerosis is causing DELC and patients with DELC appear to have an increased risk of CAD. It has insufficient sensitivity or specificity to be used as a diagnostic test but should be used as a valuable risk marker to be aware of whilst examining ears.