Hypertrophic cardiomyopathy (HCM) affects 1:500 individuals, and in majority of cases, a mutation in sarcomere proteins can explain the disease. Phenotype is heterogeneous and thus the prognosis. Many patients suffer from dyspnoea, especially at exercise. Unfortunately, sudden cardiac death (SCD) does occur at all ages and is a major cause of death in young adults. There is no proven pharmacological treatment to reduce hypertrophy or fibrosis, but beta-blockers are first-line treatment. In patients with obstruction, myectomy is preferred in the young, but in older patients, alcohol septal ablation is tried to reduce symptoms and possibly prognosis. Risk stratification of sudden cardiac death is challenging. The major established risk factors are extreme myocardial thickness, non-sustained ventricular tachycardia, unexplained syncope, abnormal exercise blood pressure response, and family history of sudden cardiac death. In 2014, a novel risk calculator was developed that also takes age, outflow gradient, and left atrial seize into account. Implantable defibrillator treatment is effective in HCM, but complications requiring surgery and inappropriate shocks remain a problem.