Background: Nephrolithiasis and nephrocalcinosis is uncommon in children; however, its incidence is increasing. Patients and Methods: A multicenter retrospective study of the clinical presentation, etiology, and outcome of childhood nephrolithiasis and compare it with nephrocalcinosis.Results: The study included 144 children; 93 with nephrolithiasis (formation of stones within renal pelvis or tubular lumen) and 51 with nephrocalcinosis. (deposition of calcium in the renal parenchyma) Mean age at presentation were 72 months and 54 months for nephrolithiasis and nephrocalcinosis, respectively. In 64.8% of the nephrolithiasis and 76% of nephrocalcinosis cases, a history of consanguinity was found. Congenital anomalies of the kidneys and urinary tract were present in 28% and 9.8% of those with nephrolithiasis and nephrocalcinosis, respectively. The most common symptoms of nephrolithiasis were flank pain (29%), hematuria (15%), and dysuria (11%). Urinary tract infection was the primary presentation in the nephrocalcinosis group (18%) followed by failure to thrive (16%), polyuria (12%), and dehydration (12%).The majority of nephrolithiasis cases were caused by metabolic disorders. In contrast, the most common underlying disorders for nephrocalcinosis were familial hypomagnesemia hypercalciuria nephrocalcinosis (35%), distal renal tubular acidosis (23%), and Bartter syndrome (6%).Clinical outcomes were significantly better in children with nephrolithiasis than those with nephrocalcinosis who had radiological evidence of worsening/persistent calcinosis and progressed more frequently to chronic kidney disease (stage II-IV) and end stage kidney disease.Conclusion: The etiology of nephrolithiasis can be identified in many children. Nephrocalcinosis is associated with worse clinical outcomes related to kidney function and disease resolution as compared to nephrolithiasis.