To study the etiological profile and patterns of clinical presentations of urolithiasis (UL) in children. This observational study included patients <18 y with UL, who were referred to the pediatric nephrology clinic. Clinical features, family history, consanguinity and estimated glomerular filtration rate (eGFR) at presentation and follow-up were recorded. The children were evaluated using relevant blood and urine investigations. A total of 72 children with UL were evaluated for the study. The etiology of UL (n = 72) included hyperoxaluria (n = 25; 34.7%), idiopathic hypercalciuria (n = 21; 29.2%), idiopathic hyperuricosuria (n = 3; 4.2%), cystinuria (n = 3; 4.2%), urate transporter defect (n = 2; 2.8%) and mixed stones (predominant component calcium oxalate) (n = 9; 12.5%). No etiology was detected in 4 cases (5.5%). Common presenting complaints included flank pain (n = 41; 56.7%), hematuria (n = 29; 40.3%), urinary tract infection (UTI) (n = 29; 40.3%) and vomiting (n = 11; 15.3%). The median age of presentation was 60 (36, 96) mo. Family history and consanguinity were present in 30 cases (41.7%) and 28 cases (38.9%) respectively. Stone analysis was done in 20 cases, of which 9 cases were mixed stones (predominant calcium oxalate) and 6 were calcium oxalate stones. Among children with urolithiasis, hyperoxaluria, idiopathic hypercalciuria, idiopathic hyperuricosuria, and cystinuria were the predominant identifiable entities, together accounting for 72% of cases; and renal colic, hematuria and UTI were the commonest clinical complaints.