Arrhythmogenic right ventricular cardiomyopathy (ARVC) is predominantly a genetically determined heart muscle disorder that is characterized by fibro-fatty replacement of the right ventricular (RV) myocardium.(1)) The clinical spectrum of ARVC may represent from asymptomatic premature ventricular complexes to ventricular tachycardia (VT) and sudden cardiac death (SCD). It is a well-known leading cause of SCD in young adults.(2,3))There is no general consensus on the management of ARVC in pregnancy, and the preferred mode of delivery is uncertain. Herein, we report a case of ARVC diagnosed at 20 weeks of gestation following a sustained VT and treated with an implantable cardiac defibrillator (ICD). We also reviewed the current knowledge and approach to ARVC in pregnancy since the literature on this condition is based on case reports.