The cardiomyopathies were previously defined as ''heart muscle diseases of unknown cause'' and were differentiated from specific heart muscle disease with known cause. With increasing understanding of etiology and pathogenesis, the difference between cardiomyopathy and specific heart muscle disease has become indistinct. The term specific cardiomyopathies are used to describe heart diseases that are associated with specific cardiac or systemic disorders. These were previously defined as specific heart muscle diseases. They included ischemic cardiomyopathy, valvular cardiomyopathy, hypertensive cardiomyopathy, inflammatory cardiomyopathy, metabolic cardiomyopathy, general system disease, muscular dystrophies, sensitivity and toxic reactions, and peripartal cardiomyopathy. The cardiomyopathies are therefore classified by the dominant pathophysiology or, if possible, by etiological/pathogenetic factors. Topol in 1985 described a syndrome called hypertensive hypertrophic cardiomyopathy that included severe concentric cardiac hypertrophy, a small left ventricular cavity, and supernormal indexes of systolic function without concurrent medical illness or ischemic heart disease. The aim of this review was to highlighted this syndrome from pathophysiological, clinical, diagnostical view and clear all the possible correlations with genetic, inflammatory, and other markers.