Right ventricular (RV) pacing can be associated with impairment of left ventricular (LV) function due to electrical dyssynchrony and myocardial remodeling (Janousek et al. in J Cardiovasc Electrophysiol 15:470–474, 2004). RV-pacing induced ventricular dysfunction is reversible by techniques such as biventricular pacing and LV apical or LV free wall pacing or turning the pacemaker off which have all been shown to restore synchrony and improve left ventricular function (Janousek et al. in J Cardiovasc Electrophysiol 15:470–474, 2004; Geldorp et al. in Heart Fail Rev 16:305–314, 2011). We describe an infant with RV-pacing induced cardiomyopathy who improved when the pacing rate was reduced thus demonstrating the relationship between pacing rate and development of LV dysfunction.