This report describes the diagnostic difficulty encountered in a young fema
le patient presenting with neurologic symptoms, atrial fibrillation and sev
ere left ventricular systolic dysfunction, eventually leading to cardiac tr
ansplantation. The scrutiny used in the evaluation of the particular aspect
of the left ventricle, and the integration of the information obtained fro
m echocardiography, angiography and magnetic resonance imaging, led to the
diagnosis of a rare and mostly unknown cause of cardiac failure. The correc
t identification of this entity is mandatory because enhanced risk of throm
boembolism. and malignant arrhythmia should be anticipated. A review of the
literature revealed only 6 patients in whom isolated non-compaction of the
left ventricle was treated by heart transplantation.