Background: Arrhythmogenic right ventricular cardiomyopathy dysplasia (ARVCD) is a common cause of sudden cardiac death among young adults and athletes. The currents study sought to evaluate clinical characteristics, echocardiographic and ECG diagnostic criteria, and follow-up results in patients with ARVCD. Methods: In the present case series, the ECG, imaging, and echocardiography records of all patients referring to our tertiary care center between 2000 and 2015 were assessed. Sex, age, cardiovascular risk factors, drug history, and family history of cardiovascular diseases were considered as the study variables. The frequency of all baseline and clinical data and the correlations between those and implantable cardioverter defibrillator (ICD) indication and survival were evaluated. Results: In this case series, 68 patients with ARVCD (mean age =39.48±15.83 y; 45 male) were evaluated. The most frequent symptom was palpitation, followed by syncope, and the most prevalent ECG findings was T-wave inversion in the precordial leads (P<0.05). Regional RV akinesia or dyskinesia was seen in 77.9%. The ICD was implanted in 55 patients: appropriate and inappropriate therapy was seen in 33 and 12 patients, respectively. The correlation between dyspnea and ICD indication was significant (P<0.05). The relationships between appropriate ICD therapy and dyspnea, peripheral edema, ascites, and severe left ventricular (LV) dysfunction were significant (P<0.05). Multivariate analysis showed that dyspnea and secondary ICD indices were the predictors of appropriate ICD therapy. The mortality rate was 11.8%. Conclusions: In our patients with ARVCD, the most common symptoms were palpitation, syncope, and T-wave inversion in the precordial leads. The correlations between appropriate ICD therapy and dyspnea, peripheral edema, ascites, and severe LV dysfunction were significant. Dyspnea and secondary ICD indication were the predictors of appropriate ICD therapy. (Iranian Heart Journal 2016; 17(1): 20-28)