Left ventricular non-compaction (LVNC) is a cardiomyopathy that can either result from arrested or abnormal myocardial morphogenesis during heart development, or can be acquired later in life. Current practice guidelines recommend different strategies for the management of patients with LVNC. Common clinical features of this disease include cardiac failure, thromboembolism, life-threatening arrhythmia or sudden cardiac death, which could indicate a worse prognosis. The disease may occur alone or in association with other congenital cardiac, neuromuscular, mitochondrial or metabolic disorders. The association of left ventricular non-compaction with other structural cardiac congenital diseases (such as atrial or ventricular septal defect, patent ductus arteriosus, obstruction of ventricular outflow tract) is rare. As clinical manifestations of LVNC are non-specific, particular imaging modalities (echocardiography, cardiovascular magnetic resonance imaging or ECG gated computed tomography) should be used in order to establish the diagnosis of LVNC. Antiarrhythmic drugs and implantable cardioverter defibrillators may be considered for the management of ventricular arrhythmias in patients with ventricular non-compaction. We report the presentation, diagnosis and management of a 46 year-old female with refractory severe cardiac failure, repeated syncope due to LVNC and atrial septal defect, requiring medical therapy and an implantable cardioverter defibrillator as a “life bridge” to heart transplantation.