A 2-year-old boy presented with an atypical renal mass with extension
into the pyelocalyceal system and distal ureter which was diagnosed in
itially as xanthogranulomatous pyelonephritis (XGP). The final diagnos
is was intrapelvic Wilms' tumor extending into the ureter. In the lite
rature, only three patients have been described with Wilms' tumor exte
nding into the ureter, none of whom had a continuous extension into th
e distal ureter.