This article reviews etiology, pathogenesis, natural history, and current treatment options in hypertrophic obstructive cardiomyopathy with a special focus on percutaneous septal ablation which has evolved as an alternative to myectomy in patients with symptoms refractory to medical treatment. Literature data and the own series of 337 interventions planned and 312 procedures completed (in-hopital mortality and pacemaker implantation rates: 1.2% [4/312] and 7% [22/312]) are discussed. Overall satisfactory clinical results were seen in about 90% of the patients treated with septal ablation.