Acromegaly is a rare disorder resulting from the excessive secretion of growth hormone (GH) and causing a specific form of cardiomyopathy. Until now, it has been widely recognized that individuals with acromegaly face an elevated risk of arrhythmias. However, high-grade atrioventricular blocks are a very rare complication of acromegaly. We report the clinical observation of a 75 years old acromegalic male presenting with high-grade atrioventricular block requiring permanent stimulation with a pacemaker.