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Introduction: Nephrocalcinosis (NC) is the increased deposition of calcium in the renal parenchyma due to different aetiologies. Aim: The present study aimed to investigate the aetiology of NC and its effect on renal function and growth in children. Materials and Methods: In the present study, 25 children diagnosed with NC between 1999 and 2016 were retrospectively analysed. Relevant demographic, clinical and laboratory data were extracted from patients’ records. Results: The median (range) age at diagnosis was 9 (3-84) months. The leading cause of NC was distal Renal Tubular Acidosis (dRTA) in 16 (64%), followed by Idiopathic Hypercalciuria (IH) in 2 (8%) children. Clinical manifestations were failure to thrive in 8 (32%), polyuria and polydipsia in 5 (20%), haematuria and renal colic in 2 (8%), urinary tract infection in 7 (28%) and in 3 (12%) cases, NC was found accidentally during routine checkup. Chronic renal insufficiency developed in two patients with dRTA and in one with hyperoxaluria. The degree of NC worsened in 3 (23%) patients, remained the same in 7 (54%) and improved in 3 (23%) patients. However, the effect of nephrocalcinosis grade change on renal function and growth was not significant. Conclusion: It is not the degree of NC rather the underlying cause and the age at diagnosis that affects the growth and renal function of children.