In the manuscript authors describe the application of rasburicase in the treatment of acute kidney injury in a child with non-malignancy associated hyperuricemia and combined congenital abnormalities. Rasburicase application was safe, well tolerated and rapidly effective. The significant fall in serum uric acid levels was accompanied by rising diuresis. The improvement of renal function prevented the need for dialysis, which was favorable for the patient and markedly reduced the costs of treatment. Rasburicase could be an option in the treatment of acute kidney injury also in children with marked hyperuricemia of non-malignancy origin.