Once reserved for heart conditions with no known cause, the term cardiomyopathy has come to signify diseases that primarily affect the myocardium whether the cause is known or not. A heterogeneous group of impairments, cardiomyopathies are characterized by abnormalities of myocardial structure and function that often lead to heart failure. As knowledge increases, classification schemes continue to evolve. Most often, cardiomyopathies are categorized depending on major structural or functional manifestations: hypertrophic, dilated, arrhythmogenic, or restrictive. The many advances in genetics over the last few decades have shown that many, if not most, cardiomyopathies are either caused directly or influenced by genetic variants. Enhanced imaging techniques allow many diagnoses to be made clinically without the risk of endomyocardial biopsy and in some cases with more accuracy. Treatment options have become more effective as well; however, these conditions are still associated with significant morbidity and mortality and are therefore challenging from a risk assessment perspective.