The trend in uric acid stone formation appears to be on the rise again throughout much of the world. This is thought secondary to diet, body habitus, and social reasons. Uric acid stone disease has a rich and fascinating medical history and probably is the oldest known stone disease. Uric acid stone disease is strongly linked to the purine metabolic pathway, and its treatment is primarily medical. Uric acid stone disease can be prevented and these are one of the few urinary tract stones that can be dissolved successfully. Surgical intervention with uric acid stone disease represents a failure of medical therapy and a whole host of modern, minimally invasive methods are available for treating patients with this disease. Finally, uric acid nephrolithiasis is associated with a variety of inborn errors of metabolism based on mutations of key enzymes in the purine metabolic pathways. This review of uric acid stone formation will start with historical consideration, review basic biochemistry, and physiology and then focus upon specific clinical scenarios. The discussions will be heavily referenced for those interested in greater details.