Left ventricular noncompaction is a poorly defined and controversial entity, with wide phenotypic expression: from a simple anatomical trait to a disease with overt cardiac affection. Current diagnostic criteria rely exclusively on morphologic features of hypertrabeculation, which have low specificity for identifying true cardiomyopathy cases. The management of left ventricular noncompaction is also heterogeneous, and there are no dedicated clinical practice guidelines. The most common cardiovascular complications are heart failure, ventricular arrhythmias, and systemic embolisms. In this review, we discuss the diagnostic limitations of the available criteria, and propose a comprehensive alternative approach (including functional imaging variables, tissue characterization, genetics, and family screening) that may help in the differential diagnosis of hypertrabeculation cases. We also describe the genetic background of the disease and discuss the overlap with other cardiomyopathies. Finally, we focus on controversial issues in clinical management and suggest the use of the previously-mentioned variables for risk stratification and for individualization of patient follow-up.