Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disorder with fibro-fatty replacement that involves the right ventricle and in the advanced phases could become biventricular. Takotsubo syndrome (TTS) is characterized by reversible systolic dysfunction occurring after a stressful event and independent of the underlying coronary artery disease. A 70-year-old female with family history of sudden cardiac death and a previous diagnosis of biventricular ARVC presented to the emergency department after experiencing chest pain and elevation of myocardial enzymes. The ECG showed sinus bradycardia and negative T-waves from V1-V3. Coronary arteries were free from stenosing atheromatous lesions at coronary angiography. Transthoracic echocardiography showed severe biventricular dysfunction due to left ventricular apical/peri-apical akinesis with apical ballooning pattern. Cardiac magnetic resonance confirmed the presence of transmural biventricular edema in the mid-apical segments in T2 weighted sequences involving both ventricles along with fibro-fatty replacement in post-contrast sequences. At discharge, a cardioverter-defibrillator was implanted. This case report shows that TTS diagnosis is challenging for the clinician due to the presence of structural cardiomyopathy with biventricular involvement. Second-line imaging modalities could be useful to identify the presence of myocardial edema and to recognize those conditions associated with poor prognosis.