A 45-year-old man with a 2-month history of breathlessness, fatigue and irregular heart rate was referred to our centre after first-ever cardiopulmonary decompensation. At presentation he was stable with heart rate of 120/min, blood pressure of 140/90 mmHg, in NYHA class II. 12-lead ECG showed atrial fi brillation, low R-waves voltage and deep S-waves with non specific ST changes in V1-V6. A posterior-anterior chest X-ray showed an enlarged cardiac silhouette, whereas on lateral picture an unusual structure was identified in the thorax cavity. An echocardiogram showed an enlarged, hypertrophied left ventricle (LV) with severe global systolic impairment. A computed tomography revealed an intestinal loop, characterized as the anterior Morgagni type congenital diaphragm hernia, in the anterior mediastinum resulting in the heart dislocation to the right side. Cardiac magnetic resonance revealed a thinned apex coupled with increased trabeculation at the apex, posterior, lateral and anterior wall. The measurements of double-layered LV wall, confi rmed the diagnosis of LV non-compaction (LVNC). This case is an illustrate of a constellation of rare anomalies of LVNC, persistent left superior vena cava with a congenital anterior Morgagni-type diaphragm hernia, causing heart dextroposition. On multidisciplinary team meeting the general decision of conservative treatment was made with typical systolic heart failure pharmacotherapy, restoration of sinus rhythm, considering referral to ICD and/or CRT-D, and regular clinical and echocardiographic follow-up of both patient and patient’s pedigree. JRCD 2012; 1: 18–23