Hypertrophic cardiomyopathy is one of the most common inherited cardiovascular disorders. It presents with a highly variable range of phenotypic expressions and an attendant small increased risk of sudden cardiac death ( SCD ), both increasing with age. Therapeutic strategies aimed at improving the patient's quality of life and longevity include pharmacological therapy, insertion of a permanent cardiac pacemaker, alcohol septal ablation, open-heart surgical myectomy, and implantation of a cardioverter defibrillator ( ICD ). This chapter reviews the genetic basis, clinical presentation, and initial management strategies for this challenging cardiac abnormality.