Preoperative diagnosis of congenital segmental giant megaureter presenting as a fetal abdominal mass.

We describe a case of congenital segmental giant megaureter in a boy that presented as a fetal abdominal mass. He also had bilateral undescended testes, bilateral vesicoureteral reflux, and segmental aniridia. He presented with hypoglycemia in the neonatal period that resolved. Postnatal magnetic resonance imaging, voiding cystourethrography and radionuclide imaging established the diagnosis, and a ureteroureterostomy was performed at 12 months.