A 9 year old boy presented with painless gross hematuria 1 month prior to admission. Ultrasound and CT scan of the whole abdomen with contrast showed a 2.6cm x 2.0cm papillary mass on the right posterolateral wall. Initial transurethral biopsy result showed negative for malignancy, fibrovascular tissue with mild chronic and acute inflammation. On repeat cystoscopy, a 4cm x 4cm pendunculated lobulated mass was located posterolateral encroaching on the right ureteral orifice. Subsequently a partial cystectomy with distal ureterectomy, right; right-to-left transureteroureterostomy with DJ stent insertion were done. Microscopic and immunostaining results support a diagnosis of rhabdoid tumor. Adjuvant chemotherapy and immunotherapy was given. On follow up, there was no evidence of recurrence.

This case emphasizes the need to follow aggressively children with gross hematuria. Biopsy should include deep biopsies as rhabdoid tumors are infiltrating tumors. Coordination with the pathologist is of extreme importance since special stainings are needed for confirmation.