Nephrolithiasis in the pediatric population is an important cause of morbidity worldwide. Presenting signs and symptoms are often considerably different from those in adults. Vague abdominal pain, hematuria, and urinary tract infection are more common in children than the classic colicky flank pain. Imaging of suspected cases should be undertaken with careful consideration of diagnostic accuracy and the potentially harmful effects of ionizing radiation. Because children with nephrolithiasis have a high chance of recurrent stone formation, a thorough risk assessment and metabolic evaluation should be performed. This review discusses the presentation, acute evaluation and risk assessment of nephrolithiasis in the pediatric population.