Recently, a novel heart syndrome, mimicking acute coronary syndrome, was described. Patients commonly present with chest pain, ST segment elevation, prolonged QT interval and slight elevation of cardiac enzymes and biomarker levels, despite the absence of significant coronary artery disease. Because of the peculiar left ventricular apical ballooning shape, given by characteristic regional wall motion abnormalities consisting in apical akinesis and basal hyperkinesis, this syndrome was named "Takotsubo" cardiomyopathy, after a round Japanese octopus fishing pot. An episode of emotional or physical stress, including a number of noncardiac illnesses, can be often identified as a trigger. Its unique features include also a strong female predominance and a complete rapid resolution. The management of this syndrome consists mainly in supportive and symptomatic therapy. The evolution, although mainly uneventful, can be complicated, rarely, by left ventricular rupture, making thus the takotsubo cardiomyopathy a newly recognized cause of sudden death. We present some data on the epidemiology, pathogenesis, clinical picture and management of this peculiar form of heart disease, which, although easily confounded with an acute coronary syndrome, has emerged as a unique nosologic entity.