The incidence of pediatric nephrolithiasis has been steadily increasing for the past several decades, with a concomitant concerning increase in health care costs and burden to children with this disease. Recent population-based studies have also demonstrated a change in the current trends of pediatric nephrolithiasis that is characterized by a significant increase in the number of girls now being affected. While changes in diet and lifestyle, obesity prevalence, and even imaging practices have been proposed to contribute to the recent increase in pediatric nephrolithiasis, a definite underlying cause remains elusive. This situation is complicated by the fact that, unlike in adults, the trends occurring in pediatric nephrolithiasis have not been studied rigorously, which contributes to the paucity of data in children. The level of concern with the increasing incidence is raised by factors unique to pediatric nephrolithiasis that could expose an affected child to more complications. Factors such as variable clinical presentation, high recurrence of kidney stones associated with abnormalities of metabolism and the urinary tract, and the possible presence of rare genetic kidney stone diseases would require physicians to comprehensively evaluate patients presenting with kidney stones. The goal of evaluation is to identify modifiable risk factors and abnormalities for which targeted therapy can be prescribed. The goals of medical and surgical treatments are to eliminate the burden of kidney stones and prevent recurrence while simultaneously minimizing complications from interventions. Patients at high risk may benefit from a specialized kidney stone clinic staffed by a pediatric nephrologist, urologist, dietitian, and clinical nurse. Such a multidisciplinary clinic can help provide the medical and surgical support needed for patients at high risk and offer key opportunities to learn more about pediatric nephrolithiasis, thereby fueling the much-needed research in this field.