A 37 year old man who presented with a cardiomyopathy, conduction defects and atrial and ventricular arrhythmias was found to have the neuromuscular manifestations of myotonic dystrophy. Despite implantation of a permanent cardiac pacemaker, antiarrhythmic drug therapy and antiarrhythmic surgery, sudden death occurred. The results of electrophysiologic studies, coronary arteriography and pathologic findings are described. This case confirms previous observations that ventricular arrhythmias, in addition to atrial arrhythmias and conduction disturbances, are cardiac manifestations of myotonic dystrophy and can lead to sudden death.