Xanthogranulomatous pyelonephritis is an uncommon form of chronic bacterial pyelonephritis characterized by the destruction of renal parenchyma and the presence of granulomas, abscesses, and collections of lipid-laden macrophages (foam cells) replacing the renal parenchyma. This case report illustrates the clinical course of bilateral diffuse xanthogranulomatous pyelonephritis with a subtle manifestation in contrast to those typically presenting with fever, flank pain or urinary tract infection. The patient therefore received supportive treatment for 18 months without hemodialysis, instead of the curative treatment, bilateral nephrectomy, which would have caused immediate loss of residual renal function and dependence on hemodialysis.