Similar to adults, stone disease in the pediatric patient may present clinically as flank/abdominal pain or hematuria. Unlike in adults, pediatric stone disease is less frequent and is often associated with an underlying metabolic disorder. Because of the 50% likelihood of finding an underlying metabolic cause for stone formation in younger children, a metabolic workup is recommended for all children with stone disease, including first-time stone formers. Stone analysis, when available, can be very helpful in determining an underlying cause. If needed, all modalities of minimally invasive surgical treatment are possible for children with stones. Surgical approaches may be needed to achieve the goal of nephron preservation. Aggressive fluid intake is the mainstay of prevention for all forms of stone disease, but specific therapy targeted to the most likely underlying metabolic abnormality is often used. Newer data are now linking stone disease to CKD, thereby emphasizing the need for a better understanding and potentially more aggressive treatment approach. With increasing frequency of stone disease in the pediatric patient and increasing survival of these patients into adulthood, the adult caregiver must become familiar with different causes and treatment approaches to stone disease in young adult patients in whom disease onset began in childhood.