The replacement of muscle by fibrous and adipose tissue lead to arrhythmogenic right ventricular dysplasia. We report the clinical features and therapeutic options of a 50 years old male with the disease followed during 12 years. The latter included pharmacological therapy surgical pseudoaneulysmal resection and radiofrequelzcy fulguration of a second arrhythmogenic focus that appeared 10 after the surgical procedure. The patient remained asymptomatic after each therapy until the disease progressed again. This follow up is one of the longest reported and documents the disease's clinical presentation evolution and treatment.