A 2 1/2-year-old girl underwent a living-related liver transplantation for biliary atresia, following which four percutaneous liver biopsies were performed uneventfully during her hospital stay for the evaluation of recurrent rejection. Her condition improved without any complications, and she was discharged from hospital in a stable condition on postoperative day (POD) 51. However, 3 months later she was readmitted with marked ascites and a moderate elevation of her liver function tests. A color Doppler ultrasound (US) confirmed hepatofugal portal blood flow, and selective celiac arteriography demonstrated an intrahepatic arterioportal (A-P) fistula. An attempt to embolize the fistula failed and a surgical approach was attempted without success. The patient died from irreversible liver failure on POD 152. A review of the previous histologic analyses revealed that arterial components were present in the fourth biopsy specimen; for this reason it was hypothesized that this procedure may have led to the development of the A-P fistula.