A 50-year-old man with hemophilia A presented with recurrent hematuria due to renal stone disease. He was receiving approximately 50,000 units of recombinant factor ( rF) VIII concentrate every year due to hematuria. Between 1996 and 2002, his serum creatinine level increased from 0.7 to 1.2 ng/ml. In an effort to resolve the problems of excessive blood loss with transfusions, recurrent rF VIII replacements and deteriorating renal function, he was offered treatment with percutaneous nephrolithotomy ( PNL) in conjunction with rF VIII administration. He underwent left PNL for left staghorn calculi in November 2002 with administration of 52,000 units rF VIII, and another PNL for the right kidney in April 2004 with the administration of 90,500 units rF VIII. A pneumatic lithotriptor was used in both operations. The serum creatinine level was 0.8 ng/ml upon completion of treatment and the patient was symptom and stone free at 10-month follow-up. He has not suffered from hematuria since that time. We conclude that bleeding disorders may not be a contraindication for PNL if corrected and monitored appropriately. Copyright (c) 2007 S. Karger AG, Basel.