BACKGROUND Paroxysmal third-degree atrioventricular block (AVB) can exhibit a vast array of symptoms, but commonly, paroxysmal AVB leads to presyncope, syncope, or possibly sudden cardiac death. We present a rare case of pause-dependent paroxysmal AVB that was triggered by a premature atrial contraction. CASE REPORT A 65-year-old man with frequent episodes of presyncope and syncope for 3 weeks was admitted to our hospital for further diagnosis. A resting 12-lead electrocardiogram showed an incomplete right bundle branch block, and a 24-h Holter recording showed multiple episodes of third-degree AVB. Intracardiac tracing revealed that the block site was distal, at the infra-His-Purkinje system. CONCLUSIONS This case highlights a rare case of pause-dependent paroxysmal AVB that was triggered by a premature atrial contraction. This type of AVB is an abrupt, unexpected, repetitive block of atrial impulses as they propagate to the ventricles. It is relatively rare, and due to its transient nature, it is often under recognized and can lead to sudden cardiac death.