We present the previously unreported CT appearance of a Wilms' tumor which extended down the ureter and protruded into the bladder as a botryoid mass. The tumor apparently arose from an intralobar nephrogenic rest and demonstrated local invasion into renal sinus vessels and papillae. There was no tumor invasion into the wall of the ureter or bladder, and therefore, the extension into the ureter and bladder did not upstage the tumor. This report adds to the list of differential diagnoses of a botryoid bladder mass in a child and demonstrates yet another unusual manifestation of Wilms' tumor.