All children less than 18 years of age who were diagnosed with a first episode of kidney stones at pediatric referral centers in Iceland during the years 1995-2000 were studied retrospectively. The diagnosis was based on clinical features and results of imaging studies. Patients were invited for evaluation at the end of the study period. Twenty-six patients (15 females, 11 males), median age 9.4 (range 0.2-14.9) years, experienced 34 episodes of kidney stones. The annual incidence was 5.6 and 6.3 per 100,000 children less than 18 and 16 years of age, respectively. Abdominal pain was the most common symptom (N=17; 69%) and urinalysis revealed hematuria in 21 patients (80.8%), sterile pyuria in 17 (65%), and 2,8-dihydroxyadeninuria in two. Six patients (23%) had positive urine cultures at the time of diagnosis and five (20%) had urinary tract anomalies. Family history of kidney stones was positive in one third of patients. Metabolic risk factors for stone formation were identified in 22 of 23 patients (96%) who underwent evaluation. Hypercalciuria, the most common metabolic risk factor, was identified in 18 patients (78%). Stones passed spontaneously in nine patients (35%) and six patients had recurrent stone episodes. The incidence of kidney stones in Icelandic children is high compared with other Western populations, affecting females more than males. Underlying metabolic risk factors were identified in most patients.