Urolithiasis is a common disease in Nephrology. Most of the stones are calcium-based stones composed of calcium phosphate or calcium oxalate, and uric acid nephrolithiasis accounts for about 10% of total stones. Chronic diarrhea, diabetes mellitus, obesity, and metabolic syndrome are risk factors of uric acid nephrolithiasis. The causes of uric acid stones including: decreased urine amount, hyperuricosuria, and urine pH less than 5.5. Computed tomography is the gold standard to diagnose urolithiasis. Urine alkalization, an increase of fluid intake, and the use of xanthine oxidase inhibitor could treat uric acid stones. Without severe complications, medical therapy is efficient in most patients with uric acid nephrolithiasis.