Hypertrophic cardiomyopathy (HCM) has a variable clinical presentation and penetrance. Many patients will have a benign course, but others will become symptomatic and develop left ventricular outflow tract obstruction. Echocardiograph remains the standard for diagnosis and assessment of obstruction, but gains are being made in the our understanding of the genetic basis of this condition. Pharmacologic therapy remains the first-line therapy in the majority of patients, but alcohol septal ablation and surgical septal myectomy may be considered in many patients. Patients should assess both the benefits and risks of each procedure based on the best available current evidence. This paper summarizes important recent developments in HCM diagnosis and therapy.