A case of persistent atrial standstill associated with muscular dystrophy of the limb girdle type is reported for the first time in the literature, In spite, of advanced myopathy, the patient delivered an apparently normal child. Hemodynamic studies including a normal coronary cinearteriography were performed in the post-partum period. The patient also had clinical and radiological evidence of peptic ulcer which may be the expression of an excessive vagal tone. A congenitial anomaly of the conduction system might explain the association of left anterior hemiblock with persistent atrial standstill, but could not be demonstrated.