Xanthogranulomatous pyelonephritis (XPN) is an atypical form of chronic pyelonephritis, characterized by the destruction of the renal parenchyma and its replacement with a chronic infiltrate of lipid-laden macrophages [1]. The clinical presentation is nonspecific and variable. Consider clear cell carcinoma, sarcomatoid renal cell carcinoma, renal tuberculosis, renal lymphoma, renal or psoas abscess, actinomycosis, renal cystic disease, and emphysematous pyelonephritis [2,3]. We present the case of a 59-year-old female patient, with a history of recurrent urinary tract infections, who came to our center due to severe anemia (5gr/dl), weight loss of 10 kg in 2 months, night sweats and weakness, concomitant sudden-onset lumbar pain and hematuria, after multiple studies, a diagnosis of PNX was made, which was confirmed by anatomopathological study.