Urate nephropathy is observed primarily in patients treated for malignancy, but several other predisposing conditions are recognized. We report a case in which urate nephropathy complicated status epilepticus and review the literature regarding previous similar cases. In addition, we discuss current views of the pathophysiology of acute kidney injury due to urate nephropathy. This case illustrates the value of carefully examining the urine of patients with acute kidney injury to identify causes that may have a specific treatment.