Xanthogranulomatous pyelonephritis is an atypical severe renal parenchymal infection, characterized macroscopically by a yellow lobulated mass, and microscopically by massive inflammatory cells and foamy hystiocytes. Preoperatively it can be confused with hypernephroma, pyonephrosis with stone or other renal inflammatory diseases. We present a case of xanthogranulomatous pyelonephritis associated with xanthogranulomatous epididymoorchitis. To our knowledge this is the first report of xanthogranulomatous pyelonephritis associated with canthogranulomatous epididymo-orchitis.