A short PQ interval is a common finding in patients with Fabry disease. However, there have been few electrophysiological studies in Fabry disease, and it is not clear whether the short PQ interval that is present in this disease results from preexcitation or enhanced atrioventricular nodal conduction. We present a case of a 43-year-old man with syncope, sick sinus syndrome, a PQ interval of 80 ms, and palpitations. Electrophysiological study showed PA, AH, and HV intervals of 24, 32, and 34 ms, respectively, and features of enhanced atrioventricular nodal conduction. The presence of an atrioventricular accessory pathway was excluded. We conclude that the short PQ interval in Fabry disease can result from accelerated conduction in the atrioventricular node.