Twelve cases of xanthogranulomatous pyelonephritis (XGPN) complicated by urolithiasis in upper tract were presented in this study. Fifty-four cases of XGPN, including 12 cases of this study, reported in China were clinically analysed in the article. All cases were with one suffered kidney, 57.4% of the cases had both XGPN and urolithiasis, 27.8% had the history of urinary infections, 99.0% had function-impaired kidney or renal failure. XGPN was frequently mis-diagnosed as renal cancer, renal tuberculosis and renal abscess etc. The diagnosis and treatment of XGPN are discussed.