Hypertrophic cardiomyopathy (HCM) is an inherited disease characterized by unexplained left ventricular hypertrophy. Although it is estimated to affect 1 out of 500 people, the HCM gene carrier prevalence is much more common, probably as high as 1 in 200 people. Most affected individuals have a normal life expectancy, whereas some patients may develop sudden cardiac death or end-stage heart failure. Despite significant developments in the treatment of HCM with surgical, interventional, and device-based procedures, the main focus of current pharmacological therapy has not evolved from the basic objectives of relief of symptoms and improvement in functional capacity. To date, no medical treatment has been shown to prolong survival or reduce the risk of sudden cardiac death. In recent decades, research focus in HCM has shifted to identify the treatments which are able to alter the natural pathophysiological process of this disease. This article reviews the currently recommended and frequently used medications (beta-blockers, nondihydropyridine calcium channel blockers, and disopyramide) and emerging pharmacological treatment options in the management of HCM. The mechanism of action and latest clinical trials of the novel agents are discussed in greater detail.