Hypertrabeculation is a feature of the left ventricle that, by itself, does not coincide with left ventricular non compaction (LVNC), which represents a specific cardiomyopathy. Nowadays, in the absence of gold standard diagnostic criteria, the clinician must integrate imaging aspects together with medical history. The family inheritance for LVNC, presence of neuromuscular disorders, symptoms or signs of heart failure, thromboembolic events, unexplained syncope, pathological findings at rest ECG, Holter ECG, stress test, systolic/diastolic dysfunction at rest echocardiogram, late gadolinium enhancement at cardiac magnetic resonance, and identification of specific mutations are all considered features useful for the diagnosis. Many aspects are not fully understood: multicenter studies, registers and observational studies are needed for a better comprehension of the pathology, adequate risk stratification and targeted follow-up.