Background: Bilateral renal agenesis is a rare congenital urinary anomaly that leads to lethal pulmonary hypoplasia. Amnioinfusion has been used to improve survival outcomes with bilateral renal agenesis. However, there is limited information regarding the bladder anatomy of these patients.

Methods: Five children diagnosed in utero with bilateral renal agenesis who received serial amnioinfusion were included. The clinical course with respect to transplantation and bladder management is described.

Results: None of the children had viable bladders on radiographic or endoscopic evaluation. Four out of five children have received renal transplantation with concurrent cutaneous ureterostomy.

Conclusions: To our knowledge, this is the first report documenting bladder anatomy in living patients born with bilateral renal agenesis. Due to the bladder anomalies, there has been no standard of management of the urinary drainage at the time of transplant. We present the bladder management strategy for five patients with bilateral renal agenesis who received care at our facility.