The metabolic syndrome is a cluster of cardiometabolic alterations at least partly dependent on reduced insulin sensitivity and hyperinsulinemia that can have several renal implications. A systematic review and meta-analysis of studies available in the international literature in English language demonstrates that the metabolic syndrome occurrence is associated with a significantly higher prevalence of nephrolithiasis (odds ratio 1.29, 95% confidence intervals: 1.11-1.51). The pathophysiological bases of this association are currently not completely understood, however. Potential pathogenetic links between the two conditions include metabolic factors that promote insulin resistance as well as stone formation in urine, environmental factors such as diet, oxidative stress and inflammation, and molecular changes impacting the transport of some analytes in urine. Metabolic syndrome-related nephrolithiasis shows peculiar clinical and biochemical characteristics and should be considered a multifactorial systemic disorder needing a multidisciplinary approach for adequate prevention and management in pediatric and adult age.