Introduction: Urolithiasis is increasingly recognized in pediatric patients and is encountered in a variety of clinical settings. Understandings of how and why stones form, along with knowledge of the pathophysiologic states that promote urinary tract calculi, provide the basis for effective clinical management. The aim of this study was to evaluate the clinical features, metabolic and anatomic risk factors in children with urolithiasis.Methods: Between 2004 and 2009, 84 children (35 girls, 49 boys) followed in our department because of urolithiasis were enrolled to participate in our study. Clinical presentation, urinary tract infection, stone localization, positive family history, stone composition, presence of anatomic abnormalities and urinary metabolic risk factors were evaluated retrospectively. Evaluation included serumbiochemistry; measurement of daily excretion of urinary calcium, uric acid, oxalate, citrate, and magnesium (in older children); and measurement of calcium, uric acid, oxalate, and creatinine in random urine samples in nontoilet-trained patients.Results: We investigated 84 patients (35 females and 49 males) with urolithiasis between 6 months and 16 years of age (mean age 5.25 ± 3.61 years). The stones’ diameter was 3.2 to 31 mm (mean7.31 ± 4.64). In 90.6% of cases the stone was located only in kidneys and in 2.4% only in bladder. The most common causes of presentation were urinary tract infection (UTI), restlessness and abdominal pain. Positive family history was detected in 27.3%, UTI in 23.8%, anatomic abnormality in 10.7% of patients. Metabolic evaluation, which was carried out in 78 patients, revealed that 104 (52.6%) of them had a metabolic riskfactor including normocalcemichypercalciuria (21.7%), Hyperuricosuria (11.5%), Cystinuria (3.8%), and Hyperoxaluria (5.1%). Anatomical malformation was found in 12 children (14.3%) including vesicoureteral reflux in 3, ureteropelvic junction stenosis in 5 and bilateral duplex system in 2, horseshoe kidney and ureterovesical junction obstruction one each.Conclusions: We think that urolithiasis remains a serious problem in children in our country. Family history of urolithiasis, urologic abnormalities (especially under the age of 5 years), metabolic disorders and urinary tract infections tend to indicate childhood urolithiasis. It is plausible to consider that better understanding of the causes of pediatric-age urolithiasis may lead to earlier diagnosis and appropriate treatment of the metabolic diseases and hence the prevention of renal damage and recurrences may be possible.