Diffuse xanthogranulomatous pyelonephritis (XGP) is a rare form of chronic infection of the child's kidney. The clinical presentation is nonspecific, and investigation often reveals a nonfunctional kidney. Preoperative diagnosis is very difficult even with ultrasonography and computed tomography. Total nephrectomy is the treatment of choice, but it is considered very difficult and is usually contraindicated for laparoscopic or retroperitoneoscopic techniques. Reported here is a case of retroperitoneal laparoscopic nephroureterectomy for XGP.