Introduction and objectives: The aim of our study was to evaluate the clinical features and to assess possible underlying aetiologic factors of pediatric urolithiasis whether metabolic, infection or structural abnormalities, with detection of any role of family history on the cause of stone development. Material and methods: The study design is a prospective study that was carried out on a total number of 66 consecutive Egyptian children diagnosed with urinary stone disease. The study was done at the department of urology at Aboul-Riche Children’s Hospital, Kasr Al-Ainy, Cairo University during the period of the study from March 2011 to April 2012. Results: The children were between 18 months to 14 years (mean, 5.864 ± 3.441 years, median 5.0 years). In 85% of the cases, the calculus was located in the upper urinary tract and in 15% it was only in the bladder. The most common presentations were dysurea, abdominal pain, and anuria. A positive family history of urinary calculi was detected in 42.4%;urinary tract infection, in 50%; and anatomic abnormality, in 9% of the patients. Metabolic evaluation revealed that 52.6% of them had a metabolic risk factor including hypercalciuria (7.5%), hyperuricosuria (4.5%) and hypomagnesuria (1.5%). Conclusions: We think that urolithiasis remains a serious problem in children in our country. Family history of urolithiasis, urologic abnormalities, metabolic disorders and urinary tract infections tend to be associated with childhood urolithiasis.