In hypertrophic cardiomyopathy (HC), ECG changes have been postulated to be an early marker of disease, detectable in sarcomere mutation carriers when LV wall thickness is still normal However, the ECG features of mutation carriers have not been fully characterized. Therefore, we systematically analyzed ECG’s in a genotyped HC population to: 1. Characterize ECG findings in mutation carriers (G+) with and without echocardiographic left ventricular hypertrophy (LVH), and 2. Evaluate the accuracy of the ECG to differentiate at- risk mutation carriers from genetically unaffected relatives during family screening. ECGs and echocardiograms were analyzed in 213 genotyped subjects (76 G+/LVH−, 57 G+/LVH+ overt HC, 80 genetically unaffected controls). Cardiac magnetic resonance imaging (CMR) was available on a subset. Q waves and repolarization abnormalities (QST) were highly specific (98%) markers for LVH− mutation carriers, present in 25% of G+/LVH− subjects and 3% of controls (p<0.001). QST ECG abnormalities remained independently predictive of carrying a sarcomere mutation after adjusting for age and impaired relaxation, another distinguishing feature of G+/LVH− subjects (OR 8.4, p=0.007). Myocardial scar or perfusion abnormalities were not detected on CMR in any G+/LVH− subjects, irrespective of ECG features. In overt HC, 75% had Q waves and/or repolarization changes but <25% demonstrated common isolated voltage criteria for LVH. In conclusion, Q waves and repolarization abnormalities are the most discriminating ECG features of sarcomere mutation carriers with and without LVH. However, due to the limited sensitivity of ECG and echocardiographic screening, genetic testing is required to definitively identify at-risk family members.