We report the clinicopathologic findings of a patient with biliary atresia associated with vesicoureteral reflux who underwent a liver transplant and nephroureterectomy simultaneously. The patient was a 22-month-old female infant born of a nonconsanguineous marriage, who was reported to be icteric from first day of life. Her antenatal history was significant for bilateral hydronephrosis. The results of a liver biopsy showed findings of biliary atresia, and the results of a radiograph showed bilateral vesicoureteral reflux. Therefore, the patient underwent a liver transplant and a right nephroureterectomy simultaneously. In the days after the operation, the patient died because of extrahepatic hematoma. In conclusion, a child having biliary atresia must remain for investigation of associated anomalies including vesicoureteral reflux.