Introduction and importance Xanthogranulomatous pyelonephritis (XPGN) is a rare pathology of the kidneys occurring in 0.6 to 1% of all cases of renal infections, in both men and women. It is characterized by severe inflammation of the renal parenchyma leading to formation of granulomatous tissue containing lipid-laden macrophages. This condition may mimic less aggressive or benign conditions but may worsen or be fatal if not treated aggressively. Case presentation Our patient is a 54 year old Caucasian female who presented with five days of left flank pain, hematuria, chills, nausea and vomiting. Imaging and biopsy results showed that the patient had XPGN. Clinical discussion XPGN is a difficult condition to diagnose as the symptoms are non-specific relative to renal cell carcinoma or other common renal infections. Definitive diagnosis is made with a biopsy; however, clues in various imaging modalities are used to make a tentative diagnosis. It is unclear whether earlier surgical intervention would have improved overall patient outcomes. Currently, a partial or complete nephrectomy is the only effective treatment. Conclusion Aggressive management including early diagnosis, antibiotics and nephrectomy appears to be critical in preventing progression and complications of XPNG.