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Urinary stones are extremely rare in the neonatal population. Most of the urinary stones in the neonate contain calcium. Nephrolithiasis in the neonate may be as urolithiasis and/or nephrocalcinosis. The incidence of nephrocalcinosis is not yet clear and can be differ in many researches due to the etiology. On the other hand, the incidence of urolithiasis in childhood is almost 10% of that in adults. Especially in the first decade, it is more common in boys. It may occur with inherited metabolic changes such as hypercalciuria, primary hyperoxaluria or cystinuria. Stone formation can be effected by iatrogenic causes such as hyperalimentation (parenteral nutrition), diuretic therapy (furosemide, acetazolamide) that is especially in the bronchopulmonary dysplasia. The stone formation may occur due to anatomic or functional obstructions and infections of urinary system. Most of the urinary stones in the neonatal period are diagnosed as a nephrocalcinosis. However there aren’t any exact differences between nephrolithiasis and nephrocalcinosis. Some authors suggested nephrocalcinosis is the initial step of urinary stone formation. On the other hand, the others purposed that both of them are different pathologies. In this review, we tried to summarized differences and similarities, in the context of urinary stones and the nephrocalcinosis in the neonates.