Treatment of pediatric urolithiasis consists of medical and surgical approaches. The main goal of the treatment is to prevent stone recurrence by avoiding multiple surgical interventions. In recent years, many innovations have been reported in the medical diagnostic evaluation protocol and in surgical treatment. According to recent reports, single mutations could be responsible for a larger proportion of renal stones. This etiologic feature holds the potential to change the management in stone prevention from metabolically directed therapy to more specific approaches. In addition, miniaturized instruments have been adopted in clinical practice. In recent years, minimally invasive endoscopic surgery is the treatment of choice in pediatric urolithiasis. This review aims to assess the current literature on medical and surgical treatment options for pediatric urolithiasis. We also aim to provide an overview of potential future advances.