ABSTRACT
A two-month old female child presenting with recurrent febrile urinary tract infection (UTI) was diagnosed to have a single-system pelvic kidney and a contralateral incomplete ureteral duplication with associated bilateral ectopic obstructive megaureters draining into the proximal urethra. Bilateral cutaneous loop ureterostomy was done at that time. Subsequently, she underwent undiversion, transuretero-pyelostomy (double left ureters to right pelvis), right ureteroneocystostomy, and augmentation ureterocystoplasty. Biopsy of the right distal ureter was consistent with megaureter with acute and chronic ureteritis. Repeat IVP post-op showed well-opacified kidneys with no signs of obstruction and a well-distended urinary bladder with moderate post-void residual urine. A high index of suspicion, coupled with meticulous physical examination and rationally combined diagnostic procedures are important to make a diagnosis of a unique combination of rare congenital urologic anomalies. (Author)