Abstract Chagas disease (CD), with approximately 10,000 deaths annually, has become a worldwide health problem. Approximately 35% of cases may show cardiac manifestations such as arrhythmias and/or conduction disorders, heart failure, thromboembolic accidents, and sudden death. The Amazon region has long been considered a non-endemic area for CD; however, in the last decades, with an increase in the number of acute and chronic cases, disease evolution has received greater attention. Here, we report the successful implementation of a cardioverter-defibrillator for the prevention of sudden death in a patient with autochthonous Chagas cardiomyopathy in the Brazilian Amazon.