Background:Children with urolithiasis have a wide range of metabolic disorders which should be carefully considered. The response to medical treatment could predict recurrence and need for surgical interventions. Objectives:The goal of this retrospective study is to determine the prevalence of metabolic disorders in Iranian children and assess patients' response to treatment based on the etiology of their urolithiasis. Materials and methods:Medical record of 100 children with urolithiasis who were referred to Bahrami hospital (affiliated hospital of Tehran University of Medical Sciences) between 2007 and 2017. Data regarding age, sex, family history, symptoms, size of the stones, type of treatments, response to treatment, and recurrence were recorded for all cases Results:Subjects had a mean age of 36 ±36 months and 62 (62%) of them were females. Twenty-two percent had a family history of urolithiasis. The mean age was 37.2±35 months in the complete response group, 38±44.4 months in the partial group, and 30±33.1 months in the no response group (p=0.7). There was no significant difference regarding sex and age with treatment response (p=0.8). In the complete response group, nearly 72% of cases had stones with sizes less than 3 mm. Three cases had surgical interventions and 18 had recurrence. Conclusions:Hypercalciuria followed by hyperoxaluria were the most common metabolic disorders in Iranian children with urolithiasis. Stone size plays an important role in the response to treatment.