Renal stone disease has been regarded as an uncommon problem in children compared to adults. However, increased awareness of this problem in children may lead to early intervention preventing long-term consequences on the kidney and the urinary tract. This article reviews the epidemiology, pathogenesis, and the most common etiologies of renal stones in children. The clinical features and diagnostic and therapeutic modalities for the specific etiologies are also outlined. Using these guidelines may be helpful not only in the treatment but also in the prevention of renal stones.