Left ventricular hypertrophy (LVH) has been recognized as an early and important complication in patients with autosomal dominant polycystic kidney disease (ADPKD). LVH is associated with arrhythmias, congestive heart failure, and increased cardiac mortality. The increase in left ventricular mass is attributed to the compensatory myocardial remodeling associated with systemic hypertension, which is also highly prevalent in those with ADPKD. Several observational studies over the past decades using echocardiography have estimated the prevalence of LVH in adults to range from 20-40%. More recently, the HALT-PKD study detected an LVH prevalence of less than 4% using magnetic resonance imaging. Some of the differences may relate to the imaging modality, variations in parameters used to define LVH, or demographic differences in the study populations. Nonetheless, there likely exists a shift in the pathophysiology of LVH in patients with ADPKD. Factors that explain this evolving trend include earlier detection and treatment of hypertension, more rigorous blood pressure control, and an increased use of renin-angiotensin-aldosterone system (RAAS) antagonists. The use of RAAS inhibitors has been shown to induce regression of LVH, and this may continue to play an important role in the cardiovascular risk management in patients with ADPKD. The results of ongoing studies will help elucidate these relationships.