An asymptomatic patient with a permanent pacemaker presented with unilateral accentuated neck pulsations. Evaluation revealed tricuspid insufficiency, a massively dilated right internal jugular vein, and obstruction of the left internal jugular vein. There was no evidence of aneurysm, vascular tumor, or fistula. The most likely explanation for this constellation of findings is tricuspid regurgitation occurring in the setting of unilateral internal jugular vein occlusion. Such a combination should be considered in the differential diagnosis of asymmetrical neck pulsations in a patient with a transvenous pacemaker.