Isolated ventricular non-compaction (IVNC) is an unclassified cardiomyopathy which occurs due to a morphogenetic abnormality involving an arrest of compaction of the loose myocardial meshwork during fetal ontogenesis. Despite recent advances in knowledge, diagnosis remains problematic because of its similarity to other diseases of the myocardium and endocardium. In this report, we describe a case of IVNC and myocardial bridging. The patient had been misdiagnosed with apical hypertrophic cardiomyopathy 2 years earlier. The correct diagnosis was established by transthoracic echocardiography and confirmed by cardiac catheterization and angiocardiography.