We describe an infant with the VATER association in whom a Wilms tumor was noted when she was 5 months old. The lower pole tumor arose near the pelvis and grew into an adjacent calix producing a cystic and solid mass with numerous papillary projections resembling sarcoma botryoides. Histopathologically, the tumor was a classical Wilms tumor but it was unusual in that the papillary projections of the tumor were covered by intestinal-like epithelium, which was interpreted as metaplastic urothelium. The margins were free of tumor and the patient is being treated according to the National Wilms Tumor Study Protocol for stage I lesions. She is currently without evidence of recurrence.