Introduction: Nephrolithiasis is recognized increasingly in children and inherited metabolic diseases are identified more frequently in children than in adults. The evaluation of a child with nephrolithiasis should be directed towards identifying physicochemical, anatomic and genetic factors predisposing to nephrolithiasis. Hypercalciuria is the most common metabolic cause of pediatric urinary calculi. In children with hypercalciuria the prevalence of urolithiasis in the family is 46-69%. Methods: There is described the case of a 12-yearsold non-Greek boy with short stature and history of recurrent nephrolithiasis since the age of 4 years old without further evaluation. Endocrinology tests for GH were normal and the bone age was markedly delayed. The kidneys ultrasound showed expanded nephrolithiasis. Hypercalciuria and hypocitraturia were revealed with normal serum calcium concentrations. In 24hours urine collection, oxalate was normal. PTH was normal. Bone mineral density was decreased. Results: A dietary modification with low sodium diet and increased water uptake, in addition with thiazide diuretics and potassium citrate were recommended. In his follow-up a month later, calcium and citrate concentrations in 24hours urine collection were normal. Conclusions: Hypercalciuria (40%) and hypocitraturia (38%) are the most common risk factors for the development of urolithiasis in children. The prognosis for nephrolithiasis depends on the primary diagnosis and adherence to therapy, but recurrence rates generally are high when the condition is left untreated.