Pediatric renal stone disease is manifested as nephro/urolithiasis (UL) and/or nephrocalcinosis (NC). Compared to adults, UL in childhood is less common, and it is believed to be around 5% in industrialized countries, while the incidence of NC is even lower except for critically ill premature infants, in whom it may reach 64%. The formation of UL and NC is caused by increased concentrations of relevant solutes, and their aggregations and adherence to renal tubule cells is facilitated by factors such as urine pH, inability of natural crystallization inhibitors, stasis of urine, as well as renal tubule damage. UL is associated with significant morbidity because of pain, susceptibility to urinary tract obstruction and infections, and the necessity of surgical procedures. NC is usually asymptomatic but is frequently progressive, and leads to chronic renal failure more often than UL. Although other imaging modalities can be used in the diagnosis of renal stone disease, ultrasound has the least risk and is most cost-effective. The majority of cases of UL and NC in children is of metabolic origin; thus, they are prone to recurrence and may cause chronic renal damage. Therefore, they deserve, even after their initial presentation, a detailed metabolic evaluation. Genetic source of renal stone disease is suspected in the following conditions: early onset, familial prevalence, familial consanguinity, multiple or recurrent stones, and NC. For all UL/NC etiologies, early identification and personalized treatment of the basic disorder is of the utmost importance.