Nephrocalcinosis is a fairly common finding especially among preterm neonates. Dexamethasone and furosemide are two of the proposed risk factors for neonatal nephrocalcinosis. Furosemide is mainly used in neonates in cases of heart failure, PHVD and in CLD. Dexamethasone is used mainly in the treatment of CLD. Aiming to determine whether these two drugs are risk factors for neonatal nephrocalcinosis, in this systematic review a search was done to identify studies addressing the cause-effect relationship between the use of such drugs and nephrocalcinosis. Then, eligible studies were analyzed. Review of the included studies supports the association between both furosemide and dexamethasone therapy in neonates and the development of nephrocalcinosis. Clinicians should balance the benefits and harms of the use of these drugs in neonates. More studies should focus on the incidence, causation and prognosis of nephrocalcinosis.