Myocardial involvement and serious electrocardiographic abnormalities are rare in patients with facioscapulohumeral (FSH) muscular dystrophy. We reported a case of FSH muscular dystrophy complicated with complete A-V block. The case was that of a 48 year-old male with the complaints of exertional dyspnea. His chest X-ray showed cardiomegaly, and the electrocardiogram recorded on admission showed complete A-V block. On the His bundle electrogram, complete A-H block and prolongation of H-V intervals were noted. Therefore, a permanent pacemaker was implanted, and he has been doing well for over 10 years after implantation. Although it is well known that serious electrocardiographic abnormalities are not infrequently associated with Duchenne's progressive muscular dystrophy (DMD), there are few reports about pacing therapy for patients with muscular dystrophy. Because the daily activity of patients with rapidly progressive type of muscular dystrophy such as DMD is at a very low level, they do not require pacing therapy. Whereas in patients with the FSH type of muscular dystrophy, which is a slowly progressive type, their daily activity is maintained at a high level. Therefore, pacing therapy should be recommended if the FSH type of muscular dystrophy is accompanied with serious bradyarrhythmias.