Acute uric acid nephropathy is a kind of acute renal failure and results from uric acid crystal deposition within the collecting ducts and the distal tubules due to rapid increase of serum uric acid concentration. Hyperuricemia can be, in the relation to the underlying physiology, classified into the three categories. i.e., increased urate production, decreased uric acid excretion, or a combination of the two. It is most commonly presented in the lymphoproliferative or myeloproliferative disorders after effective cytolytic chemotherapy in the form of tumor 1ysis syndrome. But we have recently experienced a case of a 73 year-old female patient with acute lymphoblastic leukemia whose first presentation was acute uric acid nephropathy, spontaneously developed without chemotherapy and so report it with review of related literatures.