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Abstract: Background: Signal averaged electrocardiogram (SAECG) is a specific and non-invasive tool useful for arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. However, its role in risk stratification of patients with ARVC remains largely undefined. Methods: Sixty-four patients fulfilling Task Force ARVC criteria (mean age: 47±14years-old, 56% male, 50% definite ARVC) were enrolled. The baseline demographic, electrocardiographic, structural, and electrophysiological characteristics were collected. Patients with SAECG fulfilling all 3 Task Force criteria (3+ SAECG) were categorized into group 1, and those fulfilled 2 or less criterion were categorized into group 2. The study endpoints were unstable ventricular arrhythmia (VA), device detectable sustained fast VA (cycle lengths <240ms) and cardiovascular death. Results: During a mean follow-up of 21±20months, 15 primary endpoints including 12 unstable VAs and 3 device-detected fast VAs were met. One patient died of electrical storm, and one patient underwent heart transplantation. The presence of 3+ SAECG predicted malignant events in all patients with definite and non-definite ARVC (p <0.01, OR=30.5, 95% CI=2.5–373.7) and in patients with definite ARVC alone (p =0.03, OR=11.1, 95% CI=1.3–93.9). Patients diagnosed with non-definite ARVC without 3+ SAECG were free from malignant events. Conclusions: SAECG fulfilling all 3 Task Force criteria was an independent risk predictor of malignant events in ARVC patients. SAECG may play a valuable role in ARVC risk stratification. [Copyright &y& Elsevier]