An 18-month-old female child presented with fever and an abdominal mas
s, which, after ultrasonography, computed tomography, and magnetic res
onance imaging was considered to be an atypical cystic renal neoplasm.
Nephrectomy was performed. Histopathological examination demonstrated
the mass to be focal xanthogranulomatous pyelonephritis. This lesion
should be considered in the differential diagnosis of renal neoplasms
in childhood, particularly cystic Wilms' tumor or Wilms' tumor with si
gnificant intratumoral hemorrage.